The Brain Book
Abstracting and Coding Guide for
Primary Central Nervous System Tumors
DRAFT #2 September 4, 2003
SEER Program
National Cancer Institute
Cover image and excerpt below from “Phrenology: the History of Brain Localization” published in Brain
and Mind: the Electronic Magazine on Neuroscience, March, 1997. Used with permission.
Website: www.epub.org.br/cm/n01/ frenolog/frenmap.htm
Phrenology was the study of the conformation (lumps and bumps) of the skull based on the belief that
they were indicative of mental faculties and character. The pseudoscience was developed by German
physician Franz Joseph Gall around 1800. It was very popular in England during the 19th century, and
has since, of course, been completely discredited.
Gall and his followers identified 37 mental and moral faculties which they thought were represented in
the exterior surface of the skull. These faculties were divided into several spheres: intellectual,
perceptiveness, mental energy, moral faculties, love, etc. Most of the faculties dealt with abstract and
hard-to-define personality traits, such as firmness, approbativeness, cautiousness, marvelousness,
eventuality, spirituality, veneration, amativeness. etc. Other phrenological traits have modern scientific
counterparts which can be evaluated with proper psychological tests, such as constructiveness,
destructiveness, individuality, self-esteem, idealism, affection, etc.
The main result of Gall's theory was a kind of chart of the skull, which mapped the regions where the
bumps and depressions related to the 37 faculties could be palpated, measured and diagnosed. This was a
marvelous device for practitioners, and was widely used.
The authors present this image as a tongue-in-cheek model for learning another kind of abstract theory:
how to correctly capture information on the internal mysteries of the skull – tumors of the brain and the
rest of the central nervous system.
This information has been researched carefully and is as accurate as possible. However, the material in
this draft is subject to change pending medical review of the final version.
Your comments are invited on the draft of this document. Please send your comments to:
April Fritz, RHIT, CTR, SEER Program, National Cancer Institute
e-mail: [email protected].
We gratefully acknowledge the help of Peggy Adamo, RHIT, CTR; Annette Hurlbut, RHIT, CTR; Dr.
James Smirniotopoulos; and the numerous reviewers of the draft versions of this document for their
comments and contributions.
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The Brain Book
Abstracting and Coding Guidelines for Central Nervous System Tumors
PROPOSED CONTENTS
(completed, partially completed, or planned)
Introduction
Why: the new reporting law and what it covers; implementation date
What makes a benign tumor different from a malignancy?
Reportable Tumors 1
Effective Date 1
Primary Site 1
Behavior 1
Diagnostic Terminology 1
Table 1. Reportable ICD and ICD-O Codes
2
Coding Guidelines
Laterality 3
Table 2. CNS Sites for which laterality is to be coded 3
Timing 3
Reportability/Sequence Number 4
Multiple Primaries Rules 4
Table 3. Histologic Groupings to Determine Same Histology for Non-malignant Brain Tumors
5
Table 4. Number of Tumors to Report According to CNS Multiple Primaries Rules 6
Malignant Transformation 7
Date of Diagnosis 8
Anatomic Landmarks in the Central Nervous System
9
Outlines of Structures in the Central Nervous System
(Related terminology, synonyms and ICD-O-3 codes)
10
WHO Classification of CNS Tumors
17
Structure of Morphology Tables 21
Morphology tables 2
Preferred term • Synonyms • Related terms • Definition • ICD-O-3 Code • ICD-O-3 grade •
WHO grade • ICD-9, ICD-10 casefinding codes • Part(s) of CNS affected • Clinical Features •
Related syndromes • Standard treatment • Notes (epidemiology)
Other Information
Syndromes associated with CNS tumors 71
Grading systems 72
Site-specific symptoms
Infra vs. supratentorial structures
References and resources
Index of terms
ICD-O-3 morphology codes 73
Complete index of CNS terminology
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INTRODUCTION
On January 1, 2004, all cancer registrars in the United States begin identifying and abstracting benign and
borderline tumors of the central nervous system. This Abstracting and Coding Guide for Central Nervous
System Tumors has been prepared in response to many questions about how these tumors should be
reported in cancer registries.
These changes are being implemented simultaneously by the National Program of Cancer Registries of
the Centers for Disease Control and Prevention; the Surveillance, Epidemiology and End Results (SEER)
Program of the National Cancer Institute, and in facilities in the Cancer Approvals Program of the
American College of Surgeons’ Commission on Cancer. One of the issues with the case reportability
changes is that registrars are largely unfamiliar with many of these benign and borderline tumors, the
anatomy of the central nervous system, and all the different names for individual CNS structures. This
book does not include all of the benign and borderline tumors that may arise in the central nervous
system; however it does cover the majority of tumors that are primary in the tissues of the CNS.
The following conditions are NOT covered in this book:
• Hodgkin and non-Hodgkin lymphomas (M-9590 to M-9729). Although lymphomas may arise in
the brain, they are not coded, staged, or treated in the same way as primary CNS histologies.
• Hematopoietic diseases
• Germ cell tumors
• Tumors from regional sites such as skull bone that may extend into the brain
• Metastatic tumors to the brain from other areas of the body
Reportable Tumors
Effective Date
All central nervous system tumors (malignant and non-malignant) are reportable as of a January 1, 2004
diagnosis date. Malignant CNS tumors have always been reportable.
Primary Site
Public Law 107-260 defines “brain-related tumors” as:
a listed primary tumor (whether malignant or benign) occurring in any of the following sites: (I)
The brain, meninges, spinal cord, cauda equina, a cranial nerve or nerves, or any other part of the
central nervous system. (II) The pituitary gland, pineal gland, or craniopharyngeal duct.
All cancer registries in the United States have adopted this definition for reportability of brain tumors.
Table 1 lists the specific codes from the International Classification of Diseases, ninth and tenth revisions,
and the International Classification of Diseases for Oncology, third edition.
Behavior
The following definitions are used in this manual:
Malignant:
ICD-O-3 morphology codes with a fifth digit behavior code of 3 invasive
Non-malignant: ICD-O-3 morphology codes with a fifth digit behavior code of 0 benign or 1
borderline
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Diagnostic Terminology
• Tumor diagnosis (morphology) must be listed in ICD-O-3.
Specific terms EXCLUDED are:
Hypodense mass
Intracranial mass
Cysts not listed in ICD-O-3: Rathke cleft, epidermoid, colloid (of 3rd ventricle), enterogenous,
neuroglial, pineal, ependymal, branchial cleft
• Non-malignant reportable diagnoses can include the terms “neoplasm” and “tumor.”
TABLE 1. REPORTABLE ICD AND ICD-O CODES
ICD-O-2,
ICD-O-3 &
Malignant
Benign
ICD-10
Subsite*
ICD-9
ICD-9
MENINGES
C70.0
Cerebral meninges
192.1
225.2
C70.1
Spinal meninges
192.3
225.4
C70.9
Meninges, NOS
192.1
225.2
BRAIN
C71.0
Cerebrum
191.0
225.0
C71.1
Frontal lobe
191.1
225.0
C71.2
Temporal lobe
191.2
225.0
C71.3
Parietal lobe
191.3
225.0
C71.4
Occipital lobe
191.4
225.0
C71.5
Ventricle, NOS
191.5
225.0
C71.6
Cerebellum
191.6
225.0
C71.7
Brain stem
191.7
225.0
C71.8
Overlapping lesion
191.8
225.0
C71.9
Brain, NOS
191.9
225.0
SPINAL CORD, CRANIAL NERVES AND OTHER CNS
C72.0
Spinal cord
192.2
225.3
C72.1
Cauda equina
192.2
225.3
C72.2
Olfactory nerve
192.0
225.1
C72.3
Optic nerve
192.0
225.1
C72.4
Acoustic nerve
192.0
225.1
C72.5
Cranial nerve, NOS
192.0
225.1
C72.8
Overlapping lesion
192.8
225.9
C72.9
CNS, NOS
192.9
225.9
NEUROENDOCRINE AND RELATED STRUCTURES
C75.1
Pituitary gland
194.3
227.3
C75.2
Craniopharyngeal duct
194.3
227.3
(suprasellar region)
C75.3
Pineal gland
194.4
227.4
Uncertain
ICD-9
Benign
ICD-10
Uncertain
ICD-10
237.6
237.6
237.6
D32.0
D32.1
D32.9
D42.0
D42.1
D42.9
237.5
237.5
237.5
237.5
237.5
237.5
237.5
237.5
237.5
237.5
D33.0
D33.0
D33.0
D33.0
D33.0
D33.0
D33.1
D33.1
D33.2
D33.2
D43.0
D43.0
D43.0
D43.0
D43.0
D43.0
D43.1
D43.1
D43.2
D43.2
237.5
237.5
237.9
237.9
237.9
225.1
237.9
225.9
D33.4
D33.4
D33.3
D33.3
D33.3
D33.3
D33.9
D33.9
D43.4
D43.4
D43.3
D43.3
D43.3
D43.3
D43.9
D43.9
237.0
237.0
D35.2
D35.3
D44.3
D44.4
237.1
D35.4
D44.5
Note: The bones of the skull (C41.0) and spine (C41.2) are not part of the central nervous system. Nonmalignant neoplasms arising in bone and extending into the central nervous system are not
reportable.
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CODING GUIDELINES FOR CNS TUMORS
Laterality
For each of the following malignant and non-malignant primary brain and CNS tumors listed in Table 2
with a diagnosis date on or after January 1, 2004, code Laterality using codes 1- 4 or 9 (for paired sites).
Midline tumors are coded Laterality = 9. Laterality is collected for both non-malignant and malignant
tumors as a point of information, but is used only for non-malignant tumors to determine multiple
primaries.
Table 2. CNS Sites for which Laterality is to be Coded
C70.0
Cerebral meninges, NOS
C71.0
Cerebrum
C71.1
Frontal lobe
C71.2
Temporal lobe
C71.3
Parietal lobe
C71.4
Occipital lobe
C72.2
Olfactory nerve
C72.3
Optic nerve
C72.4
Acoustic nerve
C72.5
Cranial nerve, NOS
All other subsites of C70._ , C71._ and C72._ are coded Laterality = 0 (not a paired organ) regardless of
the date of diagnosis. All pituitary and pineal gland and craniopharyngeal duct tumors (C75.1 - C75.3)
are coded Laterality = 0 (not a paired site).
All primary brain and CNS tumors diagnosed prior to January 1, 2004, are coded Laterality = 0 (not a
paired site).
Timing
Malignant
• If diagnosed in the same site and with same histology within two months, then one primary.
• If diagnosed in the same site and with same histology more than two months apart, then separate
primaries.
Non-malignant
• If a new non-malignant tumor of the same histology as an earlier one is diagnosed in the same site
at any time, then one primary. In other words, non-malignant tumors that recur in the same
location are counted as the same primary regardless of the time elapsed.
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Reportability/Sequence number
Malignant
• The sequence number for the malignancy is in the range 00-35.
Non-malignant
• A primary non-malignant tumor of any of the sites specified diagnosed on or after January 1,
2004, is reportable. The sequence number for the tumor is in the range 60 – 87.
• Non-malignant tumors diagnosed before January 1, 2004 should be included in the lifetime
sequence of non-malignant and borderline tumors in the range 60-87.
• A primary non-malignant tumor of any of the sites specified diagnosed before January 1, 2004, is
not reportable unless there are specific preexisting regional or state reporting requirements.
•
The sequencing of non-malignant tumors does not affect the sequencing of malignant tumors, and
vice versa. For example, a first malignancy (sequence 00) will remain sequence 00 if followed by a
non-malignant tumor (sequence 60-87).
MULTIPLE PRIMARIES RULES
•
For malignant CNS tumors, the multiple primaries rules are the same as for all other sites (a
difference at the 3 character level of the site code or the three digit level of the morphology
code).
1. If same site, then one primary.
Example: A malignant tumor in the parietal lobe (C71.3) and a separate malignant tumor in the
frontal lobe (C71.1). Abstract as one primary.
2. If same histology (same at three digit level of morphology code) and:
a. difference between diagnosis dates is less than 2 months:
i. 1 primary (abstract) if same site
ii. 2 primaries if different site and not stated to be a recurrence or metastases
b. difference between diagnosis dates is 2 or more months (site does not matter):
i. 2 primaries unless stated to be a recurrence or metastases
3. If different histologies (different at three digit level of morphology code) and:
a. difference between diagnosis dates is less than 2 months:
i. 2 primaries if same site unless one is more specific histology
ii. 2 primaries if different site
b. difference between diagnosis dates is 2 or more months:
i. always 2 primaries
•
For non-malignant CNS tumors, a difference at the fourth character level (subsite), histology,
and laterality must be considered.
For multiple lesions in which all are non-malignant tumors
1. If different sites, then separate primaries
Example: A benign tumor in the parietal lobe (C71.3) and a separate benign tumor in the
frontal lobe (C71.1). Count and abstract as separate primaries.
Example: Meningioma of cervical spine dura (C70.1) and separate meningioma overlying
occipital lobe (C70.0, cerebral meninges). Count and abstract as separate
primaries.
Exception: If one of the subsites is non-specific (such as brain, NOS C71.9) and the other is
specific in the same 3 character category (such as C71._), count as one primary
only. For example, biopsy of the temporal lobe (C71.2) shows benign tumor and
diagnosis from CT scan states neoplasm of brain (C71.9). Report one primary
only (C71.2)
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2. If different histologies, then separate primaries. To determine whether the tumors have
different histologies, code the histology of each of the tumors and look them up in Table 3.
a. If neither histology code is in Table 3, count and abstract as one primary if codes are the
same at the three digit level.
Example: Patient has a clear cell meningioma (9538/1) of the cerebral meninges and a
separate transitional meningioma (9537/0) in another part of the same
hemisphere. Count and abstract as one primary.
b. If the two histology codes are in the same category, count as one primary.
Example: Patient has a ganglioglioma (9505/1) of the cerebellum (C71.6) and a
neurocytoma (9506/1) of the cerebellopontine angle (C71.6). Count and
abstract as one primary.
c. If the histology codes are in different categories, count and abstract as separate primaries.
Example: Patient has a choroid plexus papilloma (9390/0) of the third ventricle (C71.5)
and a chordoid glioma (9444/1) of the third ventricle (C71.5). Count and
abstract as separate primaries.
d. If one of the histologies is in Table 3 and the other is not, compare codes at the three-digit
level. If they are the same, count as one primary. If different, count as two primaries.
Example: Patient has a choroid plexus papilloma (9390/0) diagnosed by stereotactic
needle biopsy in August and at resection in September the diagnosis is
atypical choroid plexus papilloma (9390/1). Count and abstract as one
primary.
Example: Patient has a neuroepithelioma (9503/0) diagnosed in March and a
dysembryoplastic neuroepithelial tumor (9413/1) of the occipital lobe
diagnosed in July. Count and abstract as separate primaries.
3. If same site and same histology:
a. and laterality is same side, one side unknown or not applicable (see exception under A.1
above), then one primary
b. and laterality is both sides, then separate primaries
Note: Refer to Laterality coding guidelines, above
Example: Separate temporal lobe (C71.2) benign tumors on right and left sides. Count and
abstract as separate primaries.
Table 3. Histologic Groupings to Determine Same Histology for Non-malignant Brain Tumors
Gliomas (includes gliomas, astrocytomas,
9380, 9381, 9382, 9400, 9401, 9410, 9411, 9420,
astroblastomas, and glioblastomas)
9421, 9423, 9424, 9430, 9440, 9441, 9442
Subependymomas
9383, 9384
Choroid plexus neoplasms
9390
Ependymomas
9391, 9392, 9393, 9394, 9444
Neuronal and neuronal-glial neoplasms
9412, 9413, 9505, 9506
Oligodendrogliomas
9450, 9451, 9460
Note: If two histologies are in the same group in Table 3 and counted as a single primary, use
the code for the first diagnosis or the more specific histology.
•
For multiple lesions in which one is benign and one is malignant,
1. Non-malignant tumor followed by malignant tumor: separate primaries regardless of timing
2. Malignant tumor followed by a non-malignant tumor : separate primaries regardless of timing
Table 4 is a quick reference for determining single or multiple primaries on the basis of behavior, primary
site, histology, laterality and time between diagnoses.
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Key to Table 4
Histology
Same
Non-malignant tumors: refer to Table 3
Malignant tumors: same at three digit level
Different Non-malignant tumors: refer to Table 3
Malignant tumors: different at three digit level
Site
Same
Different
Non-malignant tumors: same at the four character (subsite) level
Malignant tumors: same at the three character level
Non-malignant tumors: different at the four character (subsite) level
Malignant tumors: different at the three character level
Tumor category as determined by behavior
B
Non-malignant (behavior code /0 benign or /1 borderline)
M
Malignant
Timing
NA
Elapsed time (months) between the diagnosis of the first tumor and the diagnosis of the
second tumor
Not applicable
Laterality
Same side both tumors are in the same hemisphere
Other side one tumor is in the right hemisphere and the other is in the left hemisphere
Laterality unknown one tumor has known laterality and the other tumor is not identified or is midline
2m
two primaries unless stated to be recurrent or metastatic
2h
two primaries unless one histology is a more specific histology (subtype) of the other
Interpreting the table
Line 3 example: After determining that the four digit histology code is the same for both tumors, when
the first diagnosis has a benign behavior and the second diagnosis made more than two months later has a
malignant behavior, count and abstract as two primaries regardless of laterality or primary site.
Last line example: if the first diagnosis is malignant and the second is benign, count and abstract as two
primaries regardless of the timing, location, laterality or histology.
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Table 4. Number of Tumors to Report According to CNS Multiple Primaries Rules
Same Histology
Tumor
Timing
How Many Tumors To Report
SAME SITE
DIFFERENT SITE
1st
2nd
(months)
Same
Side
Other
Side
Laterality
unknown
Same
Side
Other
Side
Laterality
unknown
B
B
NA
1
2
1
2
2
2
B
M
<2
2
2
2
2
2
2
B
M
2+
2
2
2
2
2
2
M
M
<2
1
1
1
2m
2m
2m
M
M
2+
2m
2m
2m
2m
2m
2m
M
B
NA
2
2
2
2
2
2
Different Histology
Tumor
Timing
How Many Tumors To Report
SAME SITE
DIFFERENT SITE
1st
2nd
(months)
Same
Side
Other
Side
Laterality
unknown
Same
Side
Other
Side
Laterality
unknown
B
B
NA
2
2
2
2
2
2
B
M
<2
2
2
2
2
2
2
B
M
2+
2
2
2
2
2
2
M
M
<2
2h
2h
2h
2
2
2
M
M
2+
2
2
2
2
2
2
M
B
NA
2
2
2
2
2
2
Malignant Transformation
Malignant transformation occurs when a benign or borderline tumor develops the characteristics of
malignancy, such as invasion or more frequent mitoses. This is a determination that must be made by a
pathologist based on review of slides. For registry purposes, malignant transformation or progression can
be defined as a change in the WHO grade from one WHO grade to higher WHO grade II, III or IV.
• When a benign tumor transforms to a borderline tumor, do not create a second abstract and do not
change the original histology code.
• When a benign tumor transforms to malignancy (a rare occurrence), create a second abstract for the
malignancy.
Example: Patient is diagnosed and treated for choroid plexus papilloma (9390/0) of the right lateral
ventricle. Eighteen months later, the patient is symptomatic again and a biopsy of the
same area is reported as choroid plexus carcinoma (9390/3). Count and abstract as two
primaries.
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•
When a malignant tumor transforms to a higher WHO grade, do not create a second abstract and do
not change the original histology code.
Example: Patient diagnosed and treated for low grade astrocytoma (9400/3). Eight months later,
patient returns and CT scan and stereotactic biopsy show glioblastoma multiforme
(9440/3) in the same area. Abstract as one primary (low grade astrocytoma) and note
malignant progression in remarks.
Date of Diagnosis
The diagnosis date definition is the same for non-malignant CNS tumors, malignant CNS tumors, and all
other primary cancers. The diagnosis date is the earliest date a recognized medical practitioner says the
patient has a reportable tumor, whether that diagnosis is made clinically or pathologically. For nonmalignant CNS tumors, the diagnosis may be made clinically in a physician’s office and not confirmed
histologically until years later.
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Anatomic Landmarks
in the
Central Nervous System
Illustrations from “The Central
Nervous System” in “Nervous System”
in the Anatomy and Physiology
module of the SEER training web site,
www.training.seer.cancer.gov.
Major Portions of the Brain
Corpus callosum
Septum pellucidum
The Diencephalon
Central Brain and Brain Stem
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Outlines of Structures in the Central Nervous System
Indenting indicates that the term is part of the term above it. A cross-reference (see also...) indicates the term is considered part of
more than one area of the central nervous system. An equal sign (=) indicates that the terms on the same line are synonyms. This
is only a partial list of neuroanatomy structures. Refer to an anatomy book if a term is not listed.
BASIC OUTLINE OF CENTRAL NERVOUS SYSTEM STRUCTURES WITH ICD-O-3 TOPOGRAPHY CODES
This partial, basic outline displays the bolded terms from ICD-O-3 as they relate to the more complete detailed outline that follows.
The code for a structure in the basic outline includes the structures indented below it unless they are coded otherwise in the detailed outline.
Central Nervous System C72.9
(
I. Brain C71.9
A. Brain stem C71.7
B. Cerebellum C71.6
C. Cerebral ventricles C71.5
E. Forebrain C71.0
1. Diencephalon C71.0
2. Cerebrum C71.0
b. Cerebral cortex C71.0
i. Frontal lobe C71.1
iii. Occipital lobe C71.4
iv. Parietal lobe C71.3
vi. Temporal lobe C71.2
c. Corpus callosum C71.8
II. Meninges C70.9
Cerebral meninges C70.0
Spinal meninges C70.1
III. Peripheral Nervous System C47._
D. Peripheral nerves C47._
2. Cranial nerves C72._
a. Olfactory nerve C72.2
b. Optic nerve C72.3
h. Vestibulocochlear nerve C72.4
c, d, e, f, g, j, k, l, m. Other cranial nerves C72.5
10
IV. Spinal cord C72.0
A. Cauda equina C72.1
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DETAILED OUTLINE OF STRUCTURES IN THE CENTRAL NERVOUS SYSTEM
with ICD-O-3 annotations
CENTRAL NERVOUS SYSTEM, NOS (C72.9)
I. Brain (C71.9) = encephalon
A. Brain stem (C71.7) = brainstem; truncus encephalicus [connects hemispheres to spinal cord]
1. Midbrain (C71.7) = mesencephalon
a. Quadrigeminal plate = corpora quadrigemina; tectum mesencephali
i. inferior and superior colliculus [two pairs of rounded masses on dorsal midbrain]
ii. tectal plate = tectum of midbrain; ‘roof’ of midbrain
b. Locus ceruleus = nucleus pigmentosus pontis
c. Tegmentum mesencephali = mesencephalic tegmentum; midbrain tegmentum; tegmentum
i. Cerebral aqueduct = Aqueduct of Sylvius; mesencephalic aqueduct; aqueduct of midbrain see also C.1.2
ii. Periaqueductal gray [matter] = mesencephalic central gray; midbrain central gray
iii. Red Nucleus = nucleus ruber
iv. Ventral tegmental area
v. Floor of aqueduct
2. Reticular Formation
3. Rhombencephalon = hindbrain; hindbrain vesicle
a. Medulla oblongata (C71.7) = myelencephalon
i. Olive (C71.7) = oliva; corpus olivare; inferior olive; olivary eminence; olivary body
b. Metencephalon = afterbrain
i. Cerebellum (C71.6) see also B.
• Cerebellar cortex
- Purkinje cells
• Cerebellar nuclei
- nucleus: dentatus (dentate), emboliformis, globosus, fastigii
• Cerebellopontine angle (C71.6)
• Vermis = vermis cerebelli (C71.6)
• Pyramid (C71.7) = pyramid of cerebellum; pyramis vermis
ii. Pons (C71.7) = pons cerebelli; pons variolii
• Cochlear nucleus
• Locus ceruleus
• Vestibular nuclei
- nucleus: superior, medial (Schwalbe’s), inferior, lateral vestibular (Dieter’s)
c. Raphe nuclei
4. Trigeminal nuclei
a. Trigeminal spinal nucleus, trigeminal caudal nucleus
5. Other terms: cerebral peduncle (C71.7) = crus cerebri; pedunculus cerebri; basis pedunculi
infratentorial brain (C71.7) [area of brain below the tentorium cerebelli]
B. Cerebellum (C71.6) see also A.3.b.i
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C. Cerebral ventricles (C71.5) = Foramen of Monro; intraventricular foramen
1. Cerebral aqueduct = Aqueduct of Sylvius; mesencephalic aqueduct see also A.1.c.i.
2. Choroid plexus (C71.5) = tela vasculosa; plexus choroideus; also choroid plexus of lateral (C71.5) /third (C71.5)/fourth
venticle (C71.7)
3. Ependyma (C71.5) = endyma [epithelial lining of the ventricles]
4. Fourth ventricle (C71.7) = ventricle of rhombencephalon; ventriculus quartus
5. Lateral ventricle (paired) (C71.5) = ventriculus lateralis; ventricle of cerebral hemisphere
a. Tapetum = membrana versicolor; Fielding membrane see also E.2.c.i.
6. Septum pellucidum see also E.2.j.ii.
7. Third ventricle (C71.5) = ventricle of diencephalon; ventriculus tertius; diacele
D. Limbic system = visceral brain; “fight or flight” center
1. Amygdala = amygdaloid body; amygdaloid nucleus see also E.2.a.i. [almond-shaped nuclei in medial temporal lobe]
2. Epithalamus see also E.1.a.
a. Habenula = epiphysial stalk; Habenular nuclei see also E.1.a.i.
3. Fornix (Brain) = fimbria (brain); fimbria-fornix; fornix-fimbria see also E.2.e. [arching white matter bundle connecting
mamillary bodies with hippocampal formation]
4. Cingulate gyrus = Gyrus ginguli [grey matter wrapped around corpus callosum in medial aspect of cerebral hemisphere]
5. Hippocampus (C71.2) = hippocampus major
a. Dentate gyrus = gyrus dentatus; dentate fascia; fascia dentata
i. Hippocampal mossy fibers
b. Pyramidal cells see also E.2.b.v.
6. Hypothalamus (C71.0) = lamina terminalis see also E.1.b.
7. Olfactory Pathways = anterior perforated surface; olfactory cortex; olfactory tubercule; olfactory tract;
rhinencephalon see also E.2.h.
a. Islands of Calleja = Islets of Calleja see also E.2.g.
b. Olfactory bulb = Accessory olfactory bulb see also E.2.h.i.
8. Parahippocampal gyrus = hippocampal gyrus; gyrus hippocampi; gyrus parahippocampi see also E.2.vi.•
a. Entorhinal cortex = entorhinal area see also E.2.vi.• 9. Septum of brain = septal area; septal region see also E.2.j.
a. Septal nuclei = bed nucleus of stria terminalis; septofimbrial nucleus; dorsal/lateral/medial/triangular septal nucleus;
other names see also E.2.j.i.
10. Substantia innominata see also E.2.a.iv.
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13
E. Forebrain = Prosencephalon
1. Diencephalon [bridge between hemispheres and lower brainstem]
a. Epithalamus see also D.2.
i. Habenula = epiphysial stalk; Habenular nuclei see also D.2.a.
ii. Pineal gland (C75.3) = epiphysis; pineal body; epiphysis cerebri; corpus pineale; glandula pinealis
b. Hypothalamus (C71.0) = lamina terminalis see also D.6.
i. areas: anterior (supraoptic hypothalamus), middle (medial hypothalamus; intermediate
hypothalamic region), lateral (tuberomamillary nucleus; lateral hypothalmic nucleus),
posterior (posterior hypothalamic region)
c. Subthalamus = ventral thalamus; nucleus of ansa lenticularis; nucleus of field H; zona incerta
i. Entopeduncular nucleus = nucleus entopeduncularis
ii. Subthalamic nucleus = nucleus subthalamicus
d. Thalamus (C71.0) = pulvinar; dorsal thalamus
i. Thalamic nuclei [many specific names]
e. Other terms: Pituitary gland (C75.1) = hypophysis; glandula pituitaria; pituitary; adenohypophysis (anterior lobe of
pituitary); neurohypophysis (posterior lobe of pituitary); “master gland”
Rathke pouch (C75.1) = Rathke pocket, craniopharyngeal canal; Rathke diverticulum; hypophyseal
pouch; craniobuccal pouch; neurobuccal pouch [embryologic remnant adjacent to posterior lobe of pituitary]
2. Cerebrum (C71.0) = telencephalon [terminal bulge of embryologic neural tube]
a. Basal Ganglia = basal nuclei
i. Amygdala = amygdaloid body; amygdaloid nucleus see also D.1.
ii. Corpus Striatum = striatum; lenticular nucleus; lentiform nucleus; nucleus lentiformis
• globus pallidus = pallidum; paleostriatum
• neostriatum
- caudate nucleus = caudatum
- putamen = striate body; lenticular nucleus
iii. Nucleus Accumbens
iv. Substantia Innominata see also D.10.
• Basal nucleus of Meynert = Nucleus basalis magnocellularis; Nucleus basalis of Meynert
b. Cerebral Cortex = grey matter; “nuclear region”; pallium; internal pallium; cerebral matter
i. Frontal lobe (C71.1) = lobus frontalis; frontal pole
• Motor cortex [anterior to central sulcus]
• Prefrontal cortex = rostral part of frontal lobe
• Other terms: Broca area, Broca field; motor speech center
ii. Neocortex [six layers of neurons in cerebral cortex]
iii. Occipital lobe (C71.4) = lobus occipitalis; occipital pole
• Visual cortex = striate cortex
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E. Forebrain 2. Cerebrum b. Cerebral cortex, continued
iv. Parietal lobe (C71.3) = lobus parietalis
• Somatosensory cortex
v. Pyramidal cells see also D.5.b.
vi. Temporal lobe (C71.2) = lobus temporalis; temporal cortex
• Auditory cortex
• Uncus (C71.2) = parahippocampal gyrus; hippocampal gyrus; gyrus hippocampi; gyrus
parahippocampi; uncinate gyrus; uncus gyri parahippocampalis; see also D.8.
- entorhinal cortex = entorhinal area see also D.8.
vii. Insula = Insula of Reil; Organ of Reil; Reil; lobus insularis; insular lobe; operculum
c. Corpus callosum (C71.8) [crossover point or junction between cerebral hemispheres]
i. Tapetum (C71.8) = membrana versicolor; Fielding membrane see also C.1.a.
d. Diagonal band of Broca = diagonal band; stria diagnonalis
e. Fornix (brain)= fimbria (brain); fimbria-fornix; fornix-fimbria see also D.3.
f. Internal capsule = capsula interna [where white matter from cortex funnels down into cerebral peduncle as it passes
between basal ganglia and thalamus]
g. Islands of Calleja = Islets of Calleja see also D.7.a.
h. Olfactory pathways = anterior perforated surface; olfactory cortex; olfactory tubercule; olfactory tract;
rhinencephalon see also D.7.
i. Olfactory bulb = accessory olfactory bulb see also D.7.b.
j. Septum of brain = septal area; septal region see also D.9.
i. Septal nuclei = bed nucleus of stria terminalis; septofimbrial nucleus;
dorsal/lateral/medial/triangular septal nucleus; other names see also D.9.a.
ii. Septum pellucidum see also C.6.
k. Other terms: cerebral hemisphere, longitudinal fissure; lamina terminalis; sulca (furrow, valley); lateral
sulcus (Sylvian fissue); gyrus (ridge, convolution); white matter; supratentorial brain
14
15
II. Meninges (C70.9)
Cerebral meninges (C70.0) [includes cranial dura mater, cranial pia mater, intracranial arachnoid, intracranial meninges (all C70.0)]
Spinal meninges (C70.1) [includes spinal arachnoid, spinal dura mater, spinal pia mater (all C70.1)]
A. Arachnoid (NOS, C70.9) = arachnoid mater
1. Subarachnoid space = subarachnoid cavity; spatium subarachnoideum; leptomeningeal space; cavum
subarachnoideum; cavitas subarachnoidea
a. Cisterna magna = cerebromedullary cistern; cisterna cerebellomedullaris posterior
B. Dura (NOS, C70.9) = dura mater; pachymeninx
1. Subdural space = cavum subdurale; subdural cleavage; subdural cavity; spatium subdurale; subdural cleft
C. Pia mater (NOS, C70.9) = pia
II. Meninges, continued
D. Other terms:
leptomeninges (arachnoid + pia) = leptomeninx; meninx tenuis; pia-arachnoid; piarachnoid
epidural (C72.9) = extradural (C72.9); peridural [within the spinal canal; upon, outside, or external to the dura mater; between the dura
and bone]
falx (C70.0) [curved fold of dura mater separating the major hemispheres of the brain]
falx cerebelli (C70.0) = cerebellar falx; falcula [short vertical projection of dura mater separating the hemispheres of the cerebellum
falx cerebri (C70.0) = cerebral falx [long curved vertical projection of dura mater separating the hemispheres of the cerebrum]
tentorium cerebelli (C70.0) = tentorium; cerebellar tentorium [horizontal fold of dura mater separating the cerebrum and cerebellum]
III. Peripheral Nervous System
A. Autonomic nervous system not covered in this outline
B. Ganglia, sensory not covered in this outline
C. Nerve endings not covered in this outline
D. Peripheral nerves
1. Autonomic pathways not covered in this outline
2. Cranial nerves [12 pairs of nerves attached directly to the brain and ennervating the head and neck region
a. Olfactory nerve (I) (C72.2) = cranial nerve I; CN I; first cranial nerve; nervii olfactorii
b. Optic nerve (II) (C72.3) = cranial nerve II, CN II; second cranial nerve; nervus opticus; optic tract; optic chiasm
[Note: I and II are not actually nerves, but white matter tracts of brain]
c. Oculomotor nerve (III) (C72.5) = cranial nerve III; CN III; third cranial nerve; oculomotorius; nervus oculomotorius;
motor oculi
d. Trochlear nerve (IV) (C72.5) = cranial nerve IV; CN IV; fourth cranial nerve; nervus trochlearis; pathetic nerve
e. Trigeminal nerve (V) (C72.5) = cranial nerve V; CN V; fifth cranial nerve; trifacial nerve; nervus trigeminus
f. Abducens nerve (VI) (C72.5) = cranial nerve VI; CN VI; sixth cranial nerve; abducent nerve; nervus abducens
g. Facial nerve (VII) (C72.5) = cranial nerve VII; CN VII; seventh cranial nerve; nervus facialis; motor nerve of face
h. Vestibulocochlear nerve (VIII) (C72.4) = cranial nerve VIII; CN VIII; eighth cranial nerve; statoacoustic nerve;
nervus statoacusticus; octavus; nervus acusticus; nervus vestibulocochlearis; nervus octavus; auditory nerve
(cochlear root of VIII nerve)
j. Glossopharyngeal nerve (IX) (C72.5) = cranial nerve IX, CN IX; ninth cranial nerve; nervus glossopharyngeus
k. Vagus nerve (X) (C72.5) = cranial nerve X; CN X; tenth cranial nerve; nervus vagus; vagus; pneumogastric nerve;
wandering nerve
l. Accessory nerve (XI) (C72.5) = cranial nerve XI; CN XI; eleventh cranial nerve; spinal accessory nerve; accessorius
willisii; nervus accessorius
m. Hypoglossal nerve (XII) (C72.5) = cranial nerve XII; CN XII; twelfth cranial nerve; nervus hypoglosus
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III. Peripheral Nervous System, continued
3. Schwann cells [make myelin to insulate peripheral axons]
4. Spinal nerves not covered in this outline [31 pairs originate in the spinal cord: 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, and
1 coccygeal]
IV. Spinal Cord (C72.0) = medulla spinalis; spinal marrow; chorda spinalis; ‘the cord’ [major column of nerve tissue connected to the brain,
lying within the vertebral canal, covered with meninges and surrounded by cerebrospinal fluid, from which the spinal nerves emerge]
A. Cauda equina (C72.1) [the bundle of spinal nerves arising from the bottom of the spinal cord and comprising all of the spinal nerve
roots below the first lumbar vertebra]
B. Other terms:
conus medullaris (C72.0) = medullary cone [tapering lower end of the spinal cord]
filum terminale (C72.0) = terminal filum; nervus impar; terminal thread [long strand of spinal meninges (pia mater) extending
from the extremity of the medullary cone to the inner aspect of the spinal dural sac (pial part of filum terminale; filum terminale
internum); stout strands of connective tissue attaching the spinal dural sac to the coccyx (dural part of filum terminale; coccygeal
ligament; filum terminale externum]
TERMS RELATING TO BONES
Suprasellar (C71.9) = sellar; parasellar; relating to sella turcica (C75.1) [pituitary fossa (C75.1); depression in sphenoid bone containing pituitary
gland]
Intracranial site (C71.9) [within the skull]
Cranial fossa (C71.9) [depression or hollow place in the cranium; usually refers to the base of skull]
Anterior cranial fossa (C71.9) = fossa cranii anterior; fossa cranialis anterior [anterior subdivision of the floor of the cranial cavity; supports
frontal lobes]
Middle cranial fossa (C71.9) = fossa cranii media; fossa cranialis media [middle subdivision of the floor of the cranial cavity; supports temporal
lobes and pituitary gland]
Posterior fossa (C71.9) = posterior cranial fossa; fossa cranialis posterior [posterior subdivision of the floor of the cranial cavity, holding
cerebellum, pons, and medulla oblongata]
16
Adapted from Medical Subject Headings (MeSH), National Library of Medicine, various anatomy books and medical dictionaries.
Reviewed by James G. Smirniotopoulos, MD, Chief, Department of Radiology, Uniformed Services University of the Health Sciences
WHO Classification of CNS Tumors
Note: Items checked are included in this draft. Others listed will be included in the final version, and
others not listed may be added in the final version.
TUMORS OF NEUROEPITHELIAL TISSUE
Astrocytic tumors
T9400/3 Astrocytoma, NOS
T9401/3 Anaplastic astrocytoma
T9410/3 Protoplasmic astrocytoma
T9411/3 Gemistocytic astrocytoma
T9420/3 Fibrillary astrocytoma
T9421/1 Pilocytic astrocytoma
T9424/3 Pleomorphic xanthoastrocytoma
T9440/3 Glioblastoma, NOS
T9441/3 Giant cell glioblastoma
T9442/3 Gliosarcoma
T9442/1 Gliofibroma (not in WHO classification)
T9384/1 Subependymal giant cell astrocytoma
Oligodendroglial tumors
T9450/3 Oligodendroglioma, NOS
T9451/3 Oligodendroglioma, anaplastic
Mixed gliomas
T9382/3 Mixed glioma
T9382/3 Anaplastic oligoastrocytoma
Ependymal tumors
T9391/3 Ependymoma NOS
T9391/3 Cellular ependymoma
T9391/3 Clear cell ependymoma
T9391/3 Tanycytic ependymoma
T9392/3 Anaplastic ependymoma (see also embryonal tumors)
T9393/3 Papillary ependymoma
T9394/1 Myxopapillary ependymoma
T9383/1 Subependymoma
Choroid plexus tumors
T9390/0 Choroid plexus papilloma NOS
T9390/3 Choroid plexus carcinoma
Glial tumors of uncertain origin
T9430/3 Astroblastoma
T9381/3 Gliomatosis cerebri
T9444/1 Chordoid glioma
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Neuronal and mixed neuronal-glial tumors
T9492/0 Gangliocytoma
T9493/0 Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos)
T9412/1 Desmoplastic infantile astrocytoma; Desmoplastic infantile ganglioglioma
T9413/0 Dysembryoplastic neuroepithelial tumor
T9505/1 Ganglioglioma, NOS
T9505/3 Ganglioglioma, anaplastic
9506/1 Central neurocytoma; Cerebellar liponeurocytoma
8680/1 Paraganglioma, NOS of filium terminale
Neuroblastic tumors
9522/3 Olfactory neuroblastoma; Aesthesioneuroblastoma
9523/3 Olfactory neuroepithelioma
9500/3 Neuroblastoma NOS [of the adrenal gland and sympathetic nervous system] (see also
embryonal tumors)
Pineal parenchymal tumors
T9361/1 Pineocytoma
T9362/3 Pineoblastoma; Pineal parenchymal tumor of intermediate differentiation
Embryonal tumors
9501/3 Medulloepithelioma, NOS
9392/3 Ependymoblastoma (see also ependymal tumors)
T9470/3 Medulloblastoma NOS; Melanotic medulloblastoma
9471/3 Desmoplastic medulloblastoma
9472/3 Medullomyoblastoma
T9473/3 Primitive neuroectodermal tumor, NOS [Supratentorial PNET]
9474/3 Large cell medulloblastoma
9500/3 Neuroblastoma NOS see also neuroblastic tumors)
9490/3 Ganglioneuroblastoma
9508/3 Atypical teratoid/rhabdoid tumor
TUMORS OF PERIPHERAL NERVES
Schwannoma
9560/0 Neurilemoma NOS
9560/0 Cellular neurilemmoma
9560/0 Plexiform neurilemmoma
9560/0 Melanotic neurilemmoma
Neurofibroma
9540/0 Neurofibroma NOS
9550/0 Plexiform neurofibroma
Perineurioma
9571/0 Perineurioma, NOS
9571/0 Intraneural perineurioma, NOS
9571/0 Soft tissue perineurioma, NOS
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Malignant peripheral nerve sheath tumor (MPNST)
9540/3 Malignant peripheral nerve sheath tumor
[9540/3 Epithelioid MPNST]
[9540/3 MPNST with {divergent} mesenchymal {and/or epithelial} differentiation]
[9540/3 Melanotic MPNST]
[9540/3 Melanotic psammomatous MPNST]
TUMORS OF THE MENINGES
Tumors of meningothelial cells
T9530/0 Meningioma NOS
T9530/0 Microcystic meningioma NOS
T9530/0 Secretory meningioma NOS
T9530/0 Lymphoplasmacyte-rich meningioma NOS
T9530/0 Metaplastic meningioma NOS
T9531/0 Meningothelial meningioma
T9532/0 Fibrous meningioma
T9533/0 Psammomatous meningioma
T9534/0 Angiomatous meningioma
9537/0 Transitional meningioma
9538/1 Clear cell meningioma
T9538/1 Chordoid meningioma
T9538/3 Papillary meningioma
T9538/3 Rhabdoid meningioma
9539/1 Atypical meningioma
T9530/3 [Anaplastic] Malignant meningioma
Miscellaneous neoplasms
9120/0 Hemangioma NOS
9133/1 Epitheloid hemangioendothelioma NOS
9150/1 Hemangiopericytoma
9120/3 [Hem]angiosarcoma
Primary melanocytic lesions
8728/0 Diffuse melanocytosis
8728/1 Meningeal melanocytoma
8728/3 Meningeal melanomatosis
Tumors of uncertain histogenesis
9161/1 Hemangioblastoma
TUMORS OF THE SELLAR REGION
T9350/1 Craniopharyngioma Rathke’s pouch tumor)
9351/1 Adamantinomatous craniopharyngioma
9352/1 Papillary craniopharyngioma
9582/0 Granular cell tumor of the sellar region
CNS ENDOCRINE TUMORS
T8272/0 Pituitary adenoma
T8272/3 Pituitary carcinoma
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STRUCTURE OF THE MORPHOLOGY TABLES
The table format is the same for each disease. The tables are presented in alphabetic order by major term;
for example, astrocytoma, gemistocytic follows astrocytoma, fibrillary. An index by ICD-O-3 code is
included at the end of the book. The sections of the table are:
Header
preferred name of the tumor, ICD-O-3 morphology code, and the category of the tumor
according to the WHO Classification of Tumours of the Central Nervous System.
Preferred term preferred name of the disease (boldface term in ICD-O-3)
Synonyms
other names for the same tumor, as listed in ICD-O-3 indented under the preferred term
Related terms terms that are related to the preferred term (in other words, listed under the same ICD-O3 code but not indented). In addition, this section lists other names for the tumor,
including abbreviations, gathered from reference books and Internet resources. Terms
not listed in ICD-O-3 are marked with an asterisk (*).
Definition
a brief, mostly non-technical description of the tumor
Casefinding
the casefinding codes for the tumor (where the medical records coder should have coded
the diagnosis). Casefinding codes for malignant, benign and borderline tumors of this
cell type are included. Both ICD-9-CM codes for diagnoses index casefinding and the
proposed ICD-10-CM codes for death certificate casefinding are shown.
ICD-O-3 Morphology the correct morphology (M-_ _ _ _) and behavior (/_) codes for this diagnosis
ICD-O-3 Grade the correct 6th digit code (grade/differentiation) for the tumor, assuming that there are
no other descriptors in the diagnosis to further define the tumor as well-, moderately-, or
poorly-differentiated or anaplastic. Non-malignant tumors are not graded (use code 9).
WHO Grade
the grade assigned to this tumor in the World Health Organization Classification of
Tumours of the Central Nervous System, which correlates to the prognosis of the patient.
Note that this grade code is not the same as the ICD-O-3 grade code and should not be
used to code the sixth digit of the ICD-O-3 morphology code.
Sites affected a suggested primary site code (C_ _._) if the tumor is usually associated with a specific
site, or a more general discussion of common locations, such as infra- or supratentorial
Clinical features/symptoms the signs and symptoms of this tumor prior to diagnosis. Sometimes the
location of the tumor causes specific symptoms that might be a clue to coding the correct
primary site.
Related syndromes the names of any known inherited syndromes that are associated with this specific
type of CNS tumor
Treatment
a list of methods used to treat the disease, in general order of common usage or
effectiveness. Wherever possible, treatment descriptions are as specific as possible,
especially when they apply to particular situations such as limited extent of disease.
These treatments are the most common for the tumor; other treatments may be
administered to the patient and should be included on the abstract. Ancillary treatments
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that are not coded may be mentioned in the tables, as are treatments under scientific
investigation in clinical trials. The tab for treatment also includes two check-off items to
quickly determine whether radiation therapy or systemic therapy is commonly used for
the tumor. A box is checked if the radiation or systemic therapy is usually given as part
of standard first course of treatment or if the treatment is usually administered when
surgical resection is incomplete or not technically possible. The box is not checked if the
radiation or systemic treatment mentioned is currently administered only as part of a
clinical trial. The treatments listed in this section will include:
Surgery
For most CNS tumors, the most effective treatment is resective surgery. If resection is
incomplete, if the tumor is in an inaccessible location or if the tumor involves a sensitive
area of the brain (such as sight, motor, memory, or hearing), additional treatment may be
indicated. The procedures listed are intended to cure or palliate the patient; biopsy
procedures are generally not mentioned. Refer to the site-specific surgery codes in the
SEER Code Manual or the FORDS manual for other choices.
Radiation generally refers to external beam radiation, although other techniques may be mentioned.
Code the appropriate modality as defined by the SEER Code Manual or the FORDS
manual.
Systemic therapy wherever possible, one or more specific systemic therapy (chemotherapy,
hormone therapy, or immunotherapy) agents effective for the particular type of tumor
have been listed. Where specific agents are not listed, there may have been none
mentioned in reference material. Consult SEER Book 8, Antineoplastic Drugs for further
information. In general, steroids are administered to relieve the symptoms of intracranial
pressure and edema; these should not be coded as hormone therapy. The biological
response modifiers (immunotherapy) listed may include but are not limited to
monoclonal antibodies, vaccines, or immunotherapy agents.
Notes
includes other comments regarding the tumor, such as frequency rates, incidence by age,
median age, likelihood to progress to a higher grade tumor, median survival time, and
other items of interest to registrars and data analysts. If a special stage code is indicated
for a tumor, the Collaborative Staging System code will be listed first in this section.
Other Reference Material
A resources list has been included at the end of the fascicle to identify coding, tumor, and treatment
references used in preparation of these guidelines. This information has been provided to enhance the
cancer registrar’s understanding of these tumors and is not intended to be a required part of the cancer
abstract.
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Astroblastoma
9430/3
Neuroepithelial--Neuroepithelial Tumors of Uncertain Origin
Preferred Term
Astroblastoma
Synonyms
None listed
Related terms
None listed
Definition
Rare glial (astrocytic) neoplasm that does not contain foci of conventional,
infiltrative, fibrillary or gemistocytic astrocytoma or ependymoma.
Characteristic appearance on imaging.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225.0
D33.0, 1, 2
Borderline
237.5
D43.0, 1, 2
ICD-O-3 Morphology
9430/3
ICD-O-3 Grade
9
WHO grade
No WHO grade (can range from low grade to high grade)
Sites affected
Predominantly cerebral hemispheres; less commonly in corpus callosum,
cerebellum, optic nerves, brain stem, cauda equina
Clinical features/
Symptoms
Headaches, vomiting, seizures
Related syndromes
No associated syndromes
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Gross total resection. Adjuvant radiation therapy may be indicated for
incompletely resected or high grade lesions.
Notes
Occur most frequently in young adults.
The name astroblastoma is a misnomer dating back to 1924. These tumors
are not overly astrocytic nor blastic.
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Astrocytoma
9400/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Astrocytoma, NOS
Synonyms
Astrocytic glioma, astroglioma [obs]
Related terms
Diffuse astrocytoma; diffuse astrocytoma, low grade; LGA; fibrillary
astrocytoma (9420/3–see separate table); “ordinary” astrocytoma*
Astrocytoma, low grade; Astrocytoma, well differentiated*
Cystic astrocytoma [obs]
* term not listed in ICD-O-3
Definition
Malignant, infiltrative tumor of astrocytes with tendency to progress to
higher grade astrocytomas; no mitotic activity on microscopy
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9400/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Adults: supratentorial (frontal and temporal lobes) brain stem; spinal cord
Children: pons (brainstem “glioma”)
Clinical features/
Symptoms
Seizures; problems with speech, vision, sensation, motor activity;
personality/behavior changes. The type of symptoms may help identify
the location of the tumor.
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome); multiple
enchondromatosis type 1 (Ollier disease); Turcot syndrome,
neurofibromatosis type 1 (NF1) syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Anticonvulsants if history of seizures; corticosteroids to reduce
intracranial pressure [do not code as hormone therapy]
Removal or debulking of tumor (stereotactic biopsy, cytoreductive
surgery). No proven role for chemotherapy.
Post-op external beam radiotherapy recommended as standard therapy.
High-dose volume to enhancing tumor plus a limited margin (e.g. 2cm);
total dose 50-60 Gy. Radiation dose intensification and radiation
sensitizer approaches are not recommended as standard care. Supportive
care alone is a reasonable therapeutic option in patients over 70 with a
poor performance status.
Notes
10-15% of all astrocytic brain tumors
Affects young adults (peak 30-39); mean age at diagnosis: 34; 10% of
cases are age < 20, 30% in patients over 45.
Tendency to progress to anaplastic astrocytoma and glioblastoma
(average 4-5 years)
Mean survival time after surgery: 6-8 years
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Astrocytoma, anaplastic
9401/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Anaplastic astrocytoma
Synonyms
none in ICD-O-3
Related terms
Malignant astrocytoma*; high-grade astrocytoma* (ambiguous terms also
referring to glioblastoma), AA*
* not listed in ICD-O-3
Definition
Malignant, infiltrative tumor of astrocytes midway between WHO grade II
astrocytoma and WHO grade IV glioblastoma; some increased cellularity,
pleomorphism, extensive mitotic activity, and nuclear atypia (characteristics
of anaplasia).
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9401/3
ICD-O-3 Grade
4 (‘anaplastic’)
WHO grade
III
Sites affected
Supratentorial; brain stem
Clinical features/
Symptoms
Seizures (although less common than for astrocytoma, NOS); problems with
speech, vision, sensation, motor activity; personality/behavior changes. The
type of symptoms may help identify the location of the tumor.
Related syndromes
Inherited TP53 germline mutation; multiple enchondromatosis type 1 (Ollier
disease)
Standard treatment
Likely to have:
: Radiation therapy
: Systemic therapy
Anticonvulsants if history of seizures; corticosteroids to reduce intracranial
pressure [do not code as hormone therapy]
Removal or debulking of tumor.
Chemotherapy: BCNU, CCNU, procarbazine and combination of
procarbazine, CCNU, vincristine (PVC).
Post-op external beam radiotherapy recommended as standard therapy. Highdose volume to enhancing tumor plus a limited margin (e.g. 2cm); total dose
50-60 Gy. Radiation dose intensification and radiation sensitizer approaches
are not recommended as standard care. Supportive care alone is a reasonable
therapeutic option in patients over 70 with a poor performance status.
Notes
Affects adults (peak incidence 40-49); mean age at diagnosis: 41
Least common type of astrocytoma; may represent a short-term intermediate
lesion during the transition from WHO grade II to WHO grade IV
astrocytoma; highly likely to progress to glioblastoma (average 2 years).
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Astrocytoma, desmoplastic infantile
9412/1
Neuroepithelial–Neuronal and Mixed Neuronal-glial Tumors
Preferred Term
Desmoplastic infantile astrocytoma
Synonyms
DIA
Desmoplastic infantile ganglioglioma (DIG)
Related terms
Desmoplastic cerebral astrocytoma of infancy (DCAI)
Meningocerebral astrocytoma with desmoplastic reaction*
Superficial cerebral astrocytoma*
* not listed in ICD-O-3
Definition
Rare, large cystic tumor involving superficial cerebral cortex and attached to
dura, most common in infants. Tumor consists of neoplastic astrocytes (DIA)
or astrocytes and a neuronal component (DIG) with areas of poorly
differentiated cells.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225.0
D33.0
Borderline
237.5
D43.0
ICD-O-3 Morphology
9412/1
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Supratentorial (commonly more than one lobe): frontal, parietal, temporal,
occipital (in descending frequency).
Clinical features/
Symptoms
Increasing head circumference, bulging fontanelles (infant ‘soft spot’), eyes
deviated downward; also paresis, seizures, hyperactive reflexes
Related syndromes
No related syndromes
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Gross total resection; no role for radiation in first course.
Incompletely resected tumors should be considered for clinical trials of
chemotherapy.
Notes
In WHO Classification blue book (2000), morphology code printed as
provisional 9493/0. Either desmoplastic infantile astrocytoma or
desmoplastic infantile ganglioglioma may be the term used by the pathologist
based on the differentiation pattern of the neuroepithelial component.
Most common in infants age 1-24 months; a few cases age 5-17 have been
reported. Slight male predominance.
Generally good prognosis after surgical resection.
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Astrocytoma, fibrillary
9420/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Fibrillary astrocytoma
Synonyms
Fibrous astrocytoma
Related terms
--
Definition
Variant of astrocytoma comprised of malignant fibrillary astrocytes; no
mitotic activity, necrosis or microvascular proliferation
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9420/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Adults: supratentorial (frontal and temporal lobes) brain stem; spinal cord
Children: pons (brainstem “glioma”)
Clinical features/
Symptoms
Seizures; problems with speech, vision, sensation, motor activity;
personality/behavior changes. The type of symptoms may help identify the
location of the tumor.
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome)
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Treatment is determined by tumor location and symptoms. Surgical resection
is appropriate when the tumor is located in a part of the brain that is not
functionally important, those with large tumors exerting pressure on the
brain, or those that cause seizures. Fibrillary astrocytoma is infiltrative and
may extend an inch or more from the visible edge of the tumor, thus not
every cell can be removed surgically.
Radiation therapy is controversial for low-grade astrocytomas. Although
radiation therapy may lead to longer survival, the side effects of radiation,
such as impaired thinking and memory, limit its practical use. Radiation may
be deferred in asymptomatic patients until the tumor progresses.
Notes
Most frequent histologic variant of astrocytoma
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Astrocytoma, gemistocytic
9411/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Gemistocytic astrocytoma
Synonyms
Gemistocytoma
Related terms
--
Definition
Variant of astrocytoma comprised of malignant astrocytes, predominantly
(35%) gemistocytes; no mitotic activity, necrosis or microvascular
proliferation
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9411/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Supratentorial
Clinical features/
Symptoms
Seizures; problems with speech, vision, sensation, motor activity;
personality/behavior changes. The type of symptoms may help identify the
location of the tumor.
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome)
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Anticonvulsants if history of seizures; corticosteroids to reduce intracranial
pressure [do not code as hormone therapy]
Removal or debulking of tumor; postoperative radiation therapy in most cases
Notes
Likely to progress to anaplastic astrocytoma and glioblastoma; more likely to
progess than fibrillary astrocytoma (9420/3)
5-10% of cerebral gliomas
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Astrocytoma, pilocytic
9421/1 (See note under ICD-O-3 morphology)
Neuroepithelial–Astrocytic Tumors
Preferred Term
Pilocytic astrocytoma
Synonyms
Piloid astrocytoma
Juvenile astrocytoma
Spongioblastoma, NOS [obs]
Related terms
PA*; JPA* (juvenile pilocytic astrocytoma); cystic cerebellar astrocytoma*
* not listed in ICD-O-3
Definition
Slow growing astrocytoma with circumscribed (non-infiltrating) growth
pattern occurring in children and young adults. Tumor cells have hair-like
(pilo-) appearance under a microscope. Sometimes referred to as benign,
meaning no mitosis and no necrosis.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9421/1
Note: NAACCR reporting guidelines say that pilocytic astrocytoma should
be reported to central registries as /3 and analyzed with malignant tumors.
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Adults: anywhere in neuraxis. In descending order of frequency:
optic nerve, optic chiasm/hypothalamus, thalamus and basal ganglia, cerebral
hemispheres, cerebellum, and brain stem
Children: cerebellum (C71.6)
Clinical features/
Symptoms
Epilepsy; mental changes; focal neurological deficit; hydrocephalus resulting
in nausea, vomiting, headache, or ataxia; balance or coordination difficulties.
Related syndromes
Neurofibromatosis type 1 (NF-1) (Optic nerve glioma)
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Complete surgical removal, if possible, is curative. Subtotal resection may be
followed by postoperative radiation for symptomatic residual disease.
Recommended dose: 50 to 55 Gy in 1.8 to 2 Gy fractions.
Treatment with radiation may eventually lead to malignant progression (more
rapid growth, and brain invasion).
Notes
Pilocytic astrocytomas are associated with a 90-95% 25-year survival rate,
the highest of all primary brain gliomas.
Most frequent presentation: 0-20 years. Most common glioma in children.
10% of cerebral astrocytomas; 85% of cerebellar astrocytomas.
Most commonly diagnosed brain tumor in children aged 15-19.
When reported to a North American cancer registry, code as 9421/3.
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Astrocytoma, protoplasmic
9410/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Protoplasmic astrocytoma
Synonyms
none in ICD-O-3
Related terms
--
Definition
Rare variant of astrocytoma with no mitotic activity
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9410/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Cortex: supratentorial (frontotemporal)
Clinical features/
Symptoms
Seizures
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome); multiple
enchondromatosis type 1 (Ollier disease)
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Surgical removal of as much tumor as possible (protoplasmic astrocytoma
tends to be circumscribed rather than infiltrative, so surgery is more effective
than for other types of astrocytomas).
If tumor progresses, radiotherapy and radiosurgery are options.
Notes
About 28% of infiltrating astrocytomas
In children, very similar to pilocytic astrocytoma
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Astrocytoma, subependymal giant cell
9384/1
Neuroepithelial–Astrocytic Tumors
Preferred Term
Subependymal giant cell astrocytoma
Synonyms
none in ICD-O-3
Related terms
SEGA*
Giant cell glioma*
* not listed in ICD-O
Definition
Benign, slowly growing tumor arising in the wall of the lateral ventricles and
composed of large ganglioid astrocytes; most common neoplasm in patients
with tuberous sclerosis complex (TSC)
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5
C71.5
Benign
225.0
D33.0
Borderline
237.5
D43.0
ICD-O-3 Morphology
9384/1
ICD-O-3 Grade
9
WHO grade
I; non-invasive
Sites affected
C71.5 lateral ventricles, third ventricle, foramen of Monro, inferolateral wall
of lateral ventricle
Clinical features/
Symptoms
Epilepsy; increased intracranial pressue
May be asymptomatic.
Related syndromes
Tuberous sclerosis complex
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Surgery followed by radiation. Symptomatic tumors resected by
transcallosal or transcortical route.
No role for adjuvant radiotherapy or chemotherapy.
Notes
Most common in patients age 1-20; prognosis: 3-5 years before recurrence
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Choroid plexus carcinoma
9390/3
Neuroepithelial-Choroid Plexus Tumors
Preferred Term
Choroid plexus carcinoma
Synonyms
Choroid plexus papilloma, anaplastic
Choroid plexus papilloma, malignant
Related terms
Choroid plexus papilloma (9390/0) see separate table
Atypical choroid plexus papilloma (9390/1)
Definition
Papillary neoplasm of choroid plexus (the cells that make cerebrospinal fluid)
with malignant characteristics (frequent mitoses, nuclear pleomorphism,
increased nucleus:cytoplasm ratio); most common in children
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7225.0
C71.5, 7
Benign
Borderline
237.5
D33.0, 1
D43.0, 1
ICD-O-3 Morphology
9390/3
ICD-O-3 Grade
4 if stated as anaplastic; otherwise 9
WHO grade
III
Sites affected
Ventricles: lateral (50%), fourth (40%) and third (5%). Multiple ventricles
are involved in about 5% of cases.
Clinical features/
Symptoms
Hydrocephalus resulting from blocked CSF pathways; increased intracranial
pressure, vomiting, strabism and headache
Related syndromes
Occasionally associated with Li-Fraumeni syndrome
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Gross total resection is the treatment of choice. Incomplete resection may
warrant the addition of radiation therapy or chemotherapy.
Notes
Choroid plexus tumors in general 0.4-0.6% of all brain tumors, but 2-4% of
childhood tumors and 10-20% of tumors in first year of life. 80% of all
choroid plexus tumors are age < 20.
Choroid plexus papillomas more common than choroid plexus carcinomas by
5:1.
Choroid plexus carcinomas grow rapidly and have a 5 year survival rate of
40%
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Choroid plexus papilloma
9390/0
Neuroepithelial-Choroid Plexus Tumors
Preferred Term
Choroid plexus papilloma
Synonyms
None listed
Related terms
Atypical choroid plexus papilloma (9390/1)
Choroid plexus carcinoma (9390/3) see separate table
Definition
Benign, papillary neoplasm of choroid plexus (the cells that make
cerebrospinal fluid) most common in children
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7225.0
C71.5, 7
Benign
Borderline
237.5
D33.0, 1
D43.0, 1
ICD-O-3 Morphology
9390/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Ventricles: lateral (50%), fourth (40%) and third (5%). Multiple ventricles
are involved in about 5% of cases.
Clinical features/
Symptoms
Hydrocephalus resulting from blocked CSF pathways; increased intracranial
pressure, vomiting, strabism and headache
Related syndromes
Occasionally associated with Li-Fraumeni syndrome
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Gross total resection without adjuvant treatment is usually curable. No
adjuvant treatment is recommended for children
Notes
0.4-0.6% of all brain tumors, but 2-4% of childhood tumors and 10-20% of
tumors in first year of life. 80% of all choroid plexus tumors are age < 20.
Choroid plexus papillomas more common than choroid plexus carcinomas by
5:1.
Curable by surgery; papillomas have a 5 year survival rate of up to 100%
Atypical choroid plexus papilloma may show only one malignant feature (not
enough to define tumor as malignant). No clear diagnostic criteria have been
established.
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Craniopharyngioma
9350/1
Sellar Region Tumors–Endocrine Neoplasms
Preferred Term
Craniopharyngioma (C75.2)
Synonyms
Rathke pouch tumor (C75.1)
Related terms
Rathke’s cleft cyst
Definition
Craniopharyngioma is a benign partly cystic epithelial tumor.
Rathke pouch tumor (or cyst) is a slow-growing fluid-filled cyst, thought to
be left over from the fetal stage.
Casefinding
ICD-9-CM
ICD-10-CM
Note: Rathke cleft cyst is NOT reportable
Malignant
194.3
C75.1, 2
Benign
227.3
D35.2, 3
Borderline
237.0
D44.3, 4
ICD-O-3 Morphology
9350/1
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Pituitary Gland, Rathke pouch (C75.1)
Craniopharyngeal duct (C75.2)
Clinical features/
Symptoms
Headache, vision changes, endocrine symptoms, hydrocephalus.
Related syndromes
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Craniopharyngioma
Adults: Surgery alone if totally resectable. Debulking plus radiation
therapy if unresectable (to a dose of 5,400 cGy at 180 cGy/fraction).
Children: Surgery, conventional external radiation therapy. Stereotactic
radiosurgery or intracavitary irradiation in selected cases
Rathke pouch tumor
Observation, surgery and/or radiosurgery
Notes
Usually diagnosed in childhood (age 5-14) or in the elderly (after age 50).
Craniopharyngioma accounts for 1.2% to 4.6% of all intracranial tumors, 6%
to 9% of childhood CNS tumors, and 80-90% of neoplasms arising in the
pituitary region. Arises above the pituitary gland but below the brain itself.
Most are very close to the optic nerve, making surgical removal difficult.
They may also compress the pituitary gland and the hypothalamus causing
endocrine abnormalities.
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Dysembryoplastic neuroepithelial tumor Neuroepithelial–Neuronal and Mixed Neuronal-glial Tumors
9413/0
Preferred Term
Dysembryoplastic neuroepithelial tumor
Synonyms
None listed
Related terms
DNT*, DNET*
Dysembryoplastic neuroepithelial tumor, complex form*
Dysembryoplastic neuroepithelial tumor, simple form*
* not listed in ICD-O-3
Definition
Benign glial-neuronal tumor with multinodular archichecture and associated
cortical dysplasia. Most common in cortex of children and young adults with
long history of partial seizures.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225.0
D33.0
Borderline
237.5
D43.0
ICD-O-3 Morphology
9413/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Usually supratentorial (C71.0-C71.5); most commonly temporal lobe
(C71.2); occasionally caudate nucleus and cerebellum
Clinical features/
Symptoms
Long-standing drug-resistant partial seizures, usually not associated with
focal neurologic deficits
Related syndromes
Associated (occasionally) with neurofibromatosis type 1 (NF-1)
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Gross total resection. Radiation is not indicated for first course treatment.
Notes
Most common in ages 10-29. Male predominance.
Good prognosis. Rarely recur if resected.
Two morphologic variants: simple form (single glioneuronal element) and
complex form (presence of astrocytic, oligodendrocytic and neuronal
components); code either to 9413/0.
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Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos)
Neuroepithelial--Neuronal and Mixed Neuronal-glial Tumors
9493/0
Preferred Term
Dysplastic gangliocytoma of cerebellum
Synonyms
None listed
Related terms
Lhermitte-Duclos disease*, LDD*, cerebellar granule cell hypertrophy*,
diffuse hypertrophy of the cerebellar cortex*, gangliomatosis of the
cerebellum*
* not listed in ICD-O-3
Definition
Rare, benign (very low mitotic rate) cerebellar mass composed of dysplastic
ganglion cells arising predominantly in young adults and closely associated
with Cowden disease
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.6
C71.6
Benign
225.0
D33.1
Borderline
237.5
D43.1
ICD-O-3 Morphology
9493/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Cerebellum (C71.6)
Clinical features/
Symptoms
Ataxia, increased intracranial pressure, hydrocephalus, seizures; associated
with mental retardation
Related syndromes
Cowden disease (multiple hamartoma disease: autosomal dominant genetic
disease consisting of hamartomas, dysplastic gangliocytoma of cerebellum,
verrucous skin changes, papules and fibromas of the oral mucosa, facial
trichilemmomas, hamartomatous polyps of colon, thyroid neoplasms, and
breast cancer)
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Surgical decompression (preferably total resection) of cerebellum. Shunting
to avoid hydrocephalus. No role for radiation or chemotherapy.
Notes
Most common in young adults, but has been diagnosed in patients from
infancy through age 60.
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Ependymoblastoma
9392/3
Neuroepithelial–Embryonal Tumors
Preferred Term
Anaplastic ependymoma (see separate table)
Synonyms
Ependymoblastoma
Related terms
See notes below.
Definition
Rare malignant embryonal tumor that develops in newborns and young
children.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7; 192.2
C71.5, 7; C72.0
Benign
225.0, 3237.5
D33.0, 1, 4
Borderline
D43.0, 1, 4
ICD-O-3 Morphology
9392/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Supratentorial ventricles (lateral ventricles, third ventricle)
Clinical features/
Symptoms
Age newborn to 2: increased intracranial pressure and hydrocephalus
Older children: focal neurological symptoms based on location of lesion
Related syndromes
None known
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
No standardized treatment protocol due to rarity. Gross total resection with
consideration for radiation therapy.
Notes
Very rare; aggressive natural history. Usually fatal within 6 months to 1
year.
Ependymoblastoma is part of the spectrum of primitive neuroectodermal
tumors (PNET–9473/3), but code to ependymoblastoma 9382/3.
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Ependymoma
9391/3
Neuroepithelial–Ependymal Tumors
Preferred Term
Ependymoma, NOS
Synonyms
Epithelial ependymoma
Related terms
Cellular ependymoma (very cellular without increased mitosis) (most
common)
Clear cell ependymoma (with clear perinuclear “halo”; similar to
oligodendroglioma)
Tanycytic ependymoma (with spindly bipolar elements resembling tanycytes
{tanyos=stretch}); also called fibrillar
Papillary ependymoma (9393/3) see separate table
Definition
Slow-growing tumor of ependymal cells arising from the wall of the
ventricles or spinal canal.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7; 192.2
C71.5, 7; C72.0
Benign
225.0, 3237.5
D33.0, 1, 4
Borderline
D43.0, 1, 4
ICD-O-3 Morphology
9391/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Spinal cord (C72.0), Ventricular system (C71.5, C71.7) (fourth ventricle,
lateral ventricles, third ventricle in descending order). Infratentorial and
spinal ependymomas are equal in frequency in adults; infratentorial lesions
predominate in young children.
Brain (posterior fossa): 70% children Spinal cord: 96% adults
Clinical features/
Symptoms
Infratentorial lesions: hydrocephalus, increased intracranial pressure
(headache, nausea and vomiting, dizziness); cerebellar ataxia; paresis
Supratentorial lesions: neurological deficits, seizures, increased intracranial
hypertension Spinal lesions: motor and sensory deficits
Head enlargement in children under two years
Related syndromes
Neurofibromatosis type 2 (NF-2) for spinal ependymomas
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Resective surgery; radiation therapy is sometimes used. For localized
infratentorial ependymomas, target volume is tumor bed and a safety margin.
With neuraxis dissemination, craniospinal RT followed by primary site boost.
Chemotherapy is under clinical evaluation; it has not yet been determined
whether or not chemotherapy is useful in these tumors.
Notes
All ages are susceptible. Infratentorial tumors are most common in 0-9 age
range (6-9% of primary CNS neoplasms) with second peak at 30-40 years for
spinal tumors.
5-year survival in adults: 57%; 5 year disease-free survival in children: 50%
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Ependymoma, anaplastic
9392/3
Neuroepithelial–Ependymal Tumors
Preferred Term
Anaplastic ependymoma
Synonyms
Ependymoblastoma (see separate table)
Related terms
Malignant ependymoma*
* not listed in ICD-O-3
Definition
Highly malignant glioma of ependymal cells with high mitotic activity,
accelerated growth and unfavorable clinical outcome, especially in children.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7; 192.2
C71.5, 7; C72.0
Benign
225.0, 3237.5
D33.0, 1, 4
Borderline
D43.0, 1, 4
ICD-O-3 Morphology
9392/3
ICD-O-3 Grade
4
WHO grade
III
Sites affected
Similar to grade II ependymoma: spinal cord (C72.0), ventricular system
(C71.5, C71.7) (fourth ventricle, lateral ventricles, and third ventricle)
Clinical features/
Symptoms
Infratentorial lesions: hydrocephalus, increased intracranial pressure
(headache, nausea and vomiting, dizziness); cerebellar ataxia; paresis
Supratentorial lesions: neurological deficits, seizures, increased intracranial
hypertension Spinal lesions: motor and sensory deficits
Head enlargement in children under two years
For all types of lesions, more rapid onset than for grade II ependymoma.
Related syndromes
Neurofibromatosis type 2 (NF-2) for spinal ependymomas
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection followed by external beam radiation. Combination
chemotherapy is under clinical evaluation.
Notes
Worse prognosis than grade II ependymoma
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Ependymoma, myxopapillary
9394/1
Neuroepithelial-Ependymal
Preferred Term
Myxopapillary ependymoma
Synonyms
none listed
Related terms
none listed
Definition
Slowly growing tumor of ependymal cells with predilection for lower end of
spinal cord.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.2
C72.1, 2
Benign
225.3
D33.4
Borderline
237.5
D43.4
ICD-O-3 Morphology
9493/1
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Almost exclusively at the lower end of the spinal cord in the conus/cauda
equina/filum terminale. Occasionally in cervical-thoracic spinal cord, lateral
ventricle, or parenchyma of brain.
Clinical features/
Symptoms
Infratentorial lesions: hydrocephalus, increased intracranial pressure
(headache, nausea and vomiting, dizziness); cerebellar ataxia; paresis
Supratentorial lesions: neurological deficits, seizures, increased intracranial
hypertension Spinal lesions: motor and sensory deficits
Head enlargement in children under two years
Related syndromes
Neurofibromatosis type 2 (NF-2) for spinal ependymomas
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Resective surgery with radiation to known or suspected residual tumor.
Notes
Most common in patients aged 20-40 years. More common in males
Median survival after total or parital resection exceeds 10 years.
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Ependymoma, papillary
9393/3
Preferred Term
Neuroepithelial-Ependymal Tumors
Papillary ependymoma
Synonyms
Related terms
Definition
Rare variant of ependymoma (slow-growing tumor of ependymal cells arising
from the wall of the ventricles or spinal canal).
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7; 192.2
C71.5, 7; C72.0
Benign
225.0, 3237.5
D33.0, 1, 4
Borderline
D43.0, 1, 4
ICD-O-3 Morphology
9393/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Similar to ependymoma: spinal cord (C72.0), ventricular system (C71.5,
C71.7) (fourth ventricle, lateral ventricles, third ventricle in descending
order). Infratentorial and spinal ependymomas are equal in frequency in
adults; infratentorial lesions predominate in young children.
Brain (posterior fossa): 70% children Spinal cord: 96% adults
Clinical features/
Symptoms
Infratentorial lesions: hydrocephalus, increased intracranial pressure
(headache, nausea and vomiting, dizziness); cerebellar ataxia; paresis
Supratentorial lesions: neurological deficits, seizures, increased intracranial
hypertension Spinal lesions: motor and sensory deficits
Head enlargement in children under two years
Related syndromes
Neurofibromatosis type 2 (NF-2) for spinal ependymomas
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Adults and children: resective surgery followed by radiation therapy.
Chemotherapy trials have shown limited effectiveness.
Notes
Intracranial ependymomas represent 6-9% of primary CNS neoplasms and
generally present in young children with a mean age of 4 years. These tumors
account for 30% of primary CNS neoplasms in children younger than 3 years.
Favorable survival (in descending order): spinal, supratentorial, posterior
fossa. Cerebrospinal dissemination is a sign of poor prognosis.
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Gangliocytoma
9492/0
Neuroepithelial–Neuronal and Mixed Neuronal-glial Tumors
Preferred Term
Gangliocytoma
Synonyms
None listed
Related terms
Ganglioneuroma (9490/0) see separate table
Ganglioglioma (9505/1) see separate table
Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos) (9493/0) see
separate table
Definition
Slowly growing, well-differentiated tumor solely of mature ganglion cells
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9492/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Anywhere in CNS, including temporal lobe (most common) and other parts
of cerebrum, cerebellum, brain stem, spinal cord, optic nerves, and pituitary
and pineal glands.
Clinical features/
Symptoms
Symptoms vary by site of tumor. Supratentorial tumors: seizures of long or
short duration
Related syndromes
No related syndromes
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Complete resection is often curative. Radiotherapy postoperatively is
controversial. Chemotherapy is generally not indicated as part of first line
therapy, although it should be considered in previously treated patients with
recurrence.
Notes
All ages are susceptible.
Gangliocytomas and gangliogliomas together comprise 0.4% of CNS tumors;
about 1.3% of brain tumors; and about 4% of all pediatric brain tumors.
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Ganglioglioma, NOS
9505/1
Neuroepithelial–Neuronal and Mixed Neuronal-glial Tumors
Preferred Term
Ganglioglioma, NOS
Synonyms
Glioneuroma [obs], Neuroastrocytoma [obs]
Related terms
Gangliocytoma (9492/0) see separate table
Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos) (9493/0) see
separate table
* not listed in ICD-O-3
Definition
Neoplasms consisting entirely or in part of large mature neurons in
combination with neoplastic glial cells.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9505/1
ICD-O-3 Grade
9
WHO grade
I, II
Sites affected
Supratentorial, temporal lobe (C71.2).
Clinical features/
Symptoms
Progressive seizure disorder. Strongly associated with pharmaco-resistant
seizures and temporal lobe epilepsy.
Related syndromes
No strong link to hereditary syndromes, although cases with
neurofibromatosis type 2 (NF 2) and tuberous sclerosis have been reported.
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical excision. Postoperative radiation recommended after partial
resection.
Notes
Rare, approximately 1% of all brain tumors. Occurs in children and young
adults. In two recent series, average age of patients 18 and 31.
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Ganglioglioma, anaplastic
9505/3
Neuroepithelial–Neuronal and Mixed Neuronal-glial Tumors
Preferred Term
Ganglioglioma, anaplastic
Synonyms
None in ICD-O-3
Related terms
--
Definition
Neoplasms consisting entirely or in part of large mature neurons in
combination with neoplastic glial cells (usually astrocytes)
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9505/3
ICD-O-3 Grade
IV
WHO grade
III; can be grade IV if glial component is glioblastoma
Sites affected
Supratentorial: temporal lobe, although gangliogliomas can arise throughout
CNS.
Clinical features/
Symptoms
Progressive seizure disorder. Strongly associated with pharmacoresistant
seizures and temporal lobe epilepsy.
Related syndromes
No strong link to hereditary syndromes, although cases with
neurofibromatosis type 2 and tuberous sclerosis have been reported.
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical excision. Postoperative radiation recommended after partial
resection.
Notes
Rare, less than 1% of all brain tumors. Occur in children and young adults.
Prognosis worse than for ganglioglioma, NOS (9505/1).
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Glioblastoma
9440/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Glioblastoma
Synonyms
Glioblastoma multiforme
Spongioblastoma multiforme
Related terms
GBM*
Primary glioblastoma* (see notes below)
Secondary glioblastoma* (see notes below)
WHO grade IV glioma*
* not listed in ICD-O-3
Definition
Highly malignant infiltrative astrocytoma with extensive necrosis, high
mitotic activity and marked nuclear atypia; rapid invasion of adjacent tissues.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9440/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Supratentorial (subcortical white matter of temporal, parietal, frontal and
occipital lobes in descending order of frequency); occasionally brain stem
(malignant brainstem glioma in children); rarely cerebellum or spinal cord.
Fronto-temporal presentation is typical. Rarely metastasizes to subarachnoid
space or cerebrospinal fluid.
Clinical features/
Symptoms
Epileptic seizure with non-specific neurological symptoms; rapid
development of increased intracranial pressure; headache; personality
changes
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome); familial brain
tumors (neurofibromatosis 1 or 2) (< 1% of glial tumors); Turcot syndrome HPSM2 gene
Standard treatment
Likely to have:
: Radiation therapy
: Systemic therapy
Surgery to establish a pathologic diagnosis, relieve mass effect, and, if
possible, achieve a gross total resection to facilitate adjuvant therapy.
Postoperative radiation will prolong survival; higher doses enhance survival
time.
Chemotherapy will also prolong survival. Effective agents: BCNU
(Carmustine) and CisPlatin; maximum response rates: 30-40% range. Direct
application of BCNU via a biodegradable wafer (Gliadel) left in the tumor
bed at the time of surgery: some response but no survival benefit. Another
new technique under clinical investigation in animals is clysis, the
pressurized infusion of chemotherapy via an intracranial catheter.
Post-op external beam radiotherapy recommended as standard therapy. Highdose volume to enhancing tumor plus a limited margin (e.g. 2cm); total dose
50-60 Gy. Radiation dose intensification and radiation sensitizer approaches
are not recommended as standard care. Supportive care alone is a reasonable
therapeutic option in patients over 70 with a poor performance status.
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Notes
Most frequent brain tumor (12-15% of all intracranial tumors); 50-60% of all
astrocytic brain tumors.
Peak incidence 45-60 years; mean age at diagnosis: 53.
About 2.4-7.5% of glioblastomas are truly multiple independent tumors
according to various studies.
Surgically debulked glioblastoma will typically recur within one year.
Primary glioblastoma: arises in older patients, without clinical or pathologic
evidence of pre-existing astrocytoma; short clinical history (< 3 months).
Mean length of survival for primary glioblastoma: < 1 year.
Secondary glioblastoma: arises in younger patients as progression from
diffuse WHO grade II astrocytoma or WHO grade III anaplastic astrocytoma;
progression time 1-10 years.
Primary and secondary glioblastomas may be distinct disease entities because
of their differences in age at diagnosis, response to therapy, and probable
different genetic pathways.
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Glioblastoma, giant cell
9441/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Giant cell glioblastoma
Synonyms
Monstrocellular sarcoma [obs]
Related terms
--
Definition
Variant of glioblastoma characterized by predominant bizarre, multinucleated
giant cells.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9441/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Supratentorial (subcortial in temporal and parietal lobes)
Clinical features/
Symptoms
Epileptic seizure with non-specific neurological symptoms; rapid
development of increased intracranial pressure; headache; personality
changes. Usually a short clinical history.
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome)
Standard treatment
Likely to have:
: Radiation therapy
: Systemic therapy
See Glioblastoma (9440/3)
Notes
Rare (<1% of all brain tumors); <5% of all glioblastomas
Mean age at diagnosis: 42. All age ranges affected in children and adults
Tumor is usually distinctively circumscribed and firm
Poor prognosis.
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Gliofibroma
9442/1
Neuroepithelial–Astrocytic Tumors
Preferred Term
Gliofibroma
Synonyms
none in ICD-O-3
Related terms
--
Definition
Rare neoplasm of uncertain malignancy characterized by both astrocytic and
fibroblastic components.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9442/1
ICD-O-3 Grade
9
WHO grade
(low grade); not listed in WHO Classification
Sites affected
Cerebrum, cerebellum
Clinical features/
Symptoms
Seizures
Related syndromes
None known
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Surgical resection
Notes
Note: Gliofibroma is NOT part of the WHO classification of CNS tumors.
Childhood neoplasm with favorable prognosis
World literature limited to a few case reports.
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Glioma, chordoid
9444/1
Neuroepithelial--Neuroepithelial Tumors of uncertain origin
Preferred Term
Chordoid glioma
Synonyms
Chordoid glioma of third ventricle
Related terms
None listed
Definition
Rare, slowly growing glial tumor arising in the third ventricle
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5
C71.5
Benign
225.0
D33.0
Borderline
237.5
D43.0
ICD-O-3 Morphology
9444/1
ICD-O-3 Grade
9
WHO grade
II (provisional)
Sites affected
Third ventricle (C71.5)
Clinical features/
Symptoms
Obstructive hydrocephalus (headache, nausea, ataxia). Occasionally
hypothyroidism or visual disturbances due to displacement of hypothalamus
or optic chiasm by tumor.
Related syndromes
No related syndromes.
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Complete resection may not be possible due to the location of these tumors.
Radiation therapy may be suggested for incompletely resected tumors.
Notes
Rare. Affects adults, commonly between 30 and 70.
3:1 female predominance.
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Glioma, mixed
9382/3
Neuroepithelial–Oligodendroglial Tumors
Preferred Term
Mixed glioma
Synonyms
Oligoastrocytoma
Anaplastic oligoastrocytoma
Related terms
Mixed oligo-astrocytoma; Mixed astrocytoma-oligodendroglioma*; mixed
oligodendroglioma-astrocytoma*
Code all of the following to 9382/3, mixed glioma: Mixed astrocytomaependymoma*, mixed oligodendroglioma-ependymoma*, mixed
astrocytoma-ependymoma-oligodendroglioma*, mixed medulloblastomaastrocytoma*. All are very rare and generally have the same symptoms and
treatment as astrocytomas.
* not listed in ICD-O-3
Definition
Mixed tumor containing both malignant oligodendrocytes and astrocytes.
Oligoastrocytomas have low mitotic activity. Anaplastic oligoastrocytomas
have increased cellularity, nuclear atypia, pleomorphism and mitotic activity.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9382/3
ICD-O-3 Grade
9 if mixed glioma or oligoastrocytoma; 4 if anaplastic oligoastrocytoma
WHO grade
II if mixed glioma or oligoastrocytoma
III if anaplastic oligoastrocytoma
Sites affected
Supratentorial cortex and white matter (frontal lobe 65%, then temporal 19%)
for oligoastrocytoma, 53% frontal and 38% temporal for anaplastic
oligoastrocytoma.
Clinical features/
Symptoms
Seizures (similar to other astrocytomas and oligodendrogliomas); increased
intracranial pressure: problems with speech, vision, sensation, motor activity;
personality/behavior changes. The type of symptoms may help identify the
location of the tumor. Generally a short clinical history. Symptoms of long
duration may indicate malignant progression from a pre-existing lower grade
tumor.
Related syndromes
Very scanty evidence of association with any hereditary cancer syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection; radiation therapy may be recommended for some patients
(usually incompletely resected). Chemotherapy is under clinical evaluation
for progressive tumors.
Notes
Median survival 6.3 years for oligoastrocytoma, 4.1 years for anaplastic
oligoastrocytoma.
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Gliomatosis cerebri
9381/3
Neuroepithelial--Neuroepithelial Tumors of Uncertain Origin
Preferred Term
Gliomatosis cerebri
Synonyms
None listed
Related terms
central diffuse schwannosis*, diffuse systematic overgrowth of the glial
apparatus*, diffuse glioma*, spongioblastosis*, astrocytomatosis*, diffuse
neuroblastosis*, ependymomatosis* (use of any of these terms is
discouraged)
* not listed in ICD-O-3
Definition
Diffuse glial tumor with extensive infiltration of brain involving two or more
lobes
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225.0
D33.0, 1, 2
Borderline
237.5
D43.0, 1, 2
ICD-O-3 Morphology
9381/3
ICD-O-3 Grade
9
WHO grade
III
Sites affected
Diffuse infiltration can involve cerebrum (76%), mesencephalon, thalamus,
pons, basal ganglia, cerebellum and other sites within central nervous system
Clinical features/
Symptoms
Corticospinal tract deficits, dementia, headache, seizures, increased
intracranial pressure, and many other varied symptoms
Related syndromes
No related syndromes
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
There are no effective treatments although radiation therapy may allow
quality of life to be maintained longer.
Temozolomide has been used in clinical trials with some success.
Notes
Rare tumor; little is known. In largest series, peak incidence was 40-50
years.
Poor prognosis because of diffuse cerebral involvement. Median survival
time less than 12 months.
Gliomatosis cerebri may be a term used to describe widespread recurrence
after treatment of a previous glioma. If the patient had a previous brain
tumor, be careful about determining whether the gliomatosis cerebri is a true
second primary.
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Gliosarcoma
9442/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Gliosarcoma
Synonyms
Glioblastoma with sarcomatous component
Related terms
See notes below.
Definition
Variant of glioblastoma showing both glial and mesenchymal (sarcomatous)
differentiation. The glial and sarcomatous portions of the tumor may be
intermixed or side by side.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9442/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Supratentorial (temporal, frontal, parietal, occipital in decreasing order of
frequency)
Clinical features/
Symptoms
Seizures and/or paresis (increased intracranial pressure). Short clinical
history. The type of symptoms may help identify the location of the tumor.
Related syndromes
Inherited TP53 germline mutation (Li-Fraumeni syndrome)
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
See Glioblastoma (9440/3), but even more resistant to conventional
treatments (surgery, radiation therapy and chemotherapy) than glioblastoma.
Small tumors might be partially controlled by radiosurgery.
Post-op external beam radiotherapy recommended as standard therapy. Highdose volume to enhancing tumor plus a limited margin (e.g. 2cm); total dose
50-60 Gy. Radiation dose intensification and radiation sensitizer approaches
are not recommended as standard care. Supportive care alone is a reasonable
therapeutic option in patients over 70 with a poor performance status.
Notes
About 2% of all glioblastomas.
Mean age at diagnosis: 53; peak incidence 40-60 years
The CNS tumor may be described as glial and also forming cartilage, bone,
osteoid-chondral tissue, smooth and striated muscle, or elements of
fibrosarcoma or malignant fibrous histiocytoma. Regardless of these
features, the tumor is coded to 9442/3.
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Medulloblastoma
9470/3
Embryonal Tumors
Preferred Term
Medulloblastoma, NOS
Synonyms
none in ICD-O-3
Related terms
Melanotic medulloblastoma
Classic medulloblastoma*, desmoplastic medulloblastoma (9471/3–see
separate table)
* not listed in ICD-O-3
Definition
Malignant, invasive embryonal tumor of cerebellum with neuroectodermal
differentiation.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.6
C71.6
Benign
225.0
D33.1
Borderline
237.5
D43.1
ICD-O-3 Morphology
9470/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Cerebellar vermis (C71.6) (75%), less commonly fourth ventricle (C71.5)
Children: PNET in or near a spinal fluid sac known as the fourth ventricle is
called medulloblastoma.
Clinical features/
Symptoms
Ataxia, gait disturbances, increased intracranial pressure, hydrocepahalus,
headache, vomiting, lethargy, increasing head circumference.
Related syndromes
Gorlin syndrome, Rubinstein-Taybi syndrome, Turcot syndrome
Standard treatment
Likely to have:
: Radiation therapy
: Systemic therapy
Surgical resection, radiotherapy to craniospinal field, followed by posterior
fossa boost.
Chemotherapy either pre-RT or in combination with RT: vincristine, CCNU
and cisplatin or vincristine, cyclophasphamide, and cisplatin.
Notes
Generally affects patients <20 years. More than 70% of cases <16, peak at
age 7. More common in males.
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Meningioma, NOS
9530/0
Meningeal Tumors
Preferred Term
Meningioma
Synonyms
None in ICD-O-3
Related terms
Microcystic meningioma (variant having the appearance of many small
cysts)
Secretory meningioma (variant that stains positive for carcinoembryonic
antigen; may present with prominent peritumoral edema)
Lymphoplasmacyte-rich meningioma (variant with extensive chronic
inflammatory infiltrates)
Metaplastic meningioma (variant that may display osseous, cartilaginous,
lipomatous, myxoid or xanthomatous changes–code to 9530/0)
Definition
Slowly growing, benign tumor attached to the dura mater and composed of
neoplastic meningothelial (arachnoidal) cells.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9530/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Intracranial: Parasagittal cerebral convexities, falx cerebri, sphenoid wings,
olfactory grooves, parasellar region, orbital cavity, cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Neurofibromatosis type 2; also possible links to Cowden syndrome and
Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas.
Radiation therapy may reduce recurrence rate and prolong time to recurrence.
Recommended: Postoperative external-beam doses of approximately
5,400cGy in 30 fractions given daily over 6 weeks.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Approximately 15% of primary intracranial neoplasms and 25% of primary
intraspinal neoplasms. Peak incidence ages 50-69. More common in women.
Meningiomas in children tend to be aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
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Meningioma, malignant [anaplastic]
9530/3
Meningeal Tumors
Preferred Term
Meningioma, malignant
Synonyms
Meningioma, anaplastic
Leptomeningeal sarcoma
Meningeal sarcoma
Meningothelial sarcoma
Related terms
Benign and atypical meningiomas (see separate tables)
Definition
A meningioma with malignant histological features more extensive than the
abnormalities present in atypical meningioma.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9530/3
ICD-O-3 Grade
4 if stated to be anaplastic; otherwise 9
WHO grade
III
Sites affected
90% are supratentorial. Atypical and anaplastic meningiomas are more
common on the falx and the lateral convexities than elsewhere.
Clinical features/
Symptoms
Slow growing. Can remain asypmtomatic for years. Can reach large size
without producing significan symptoms. When symptomatic, common signs
are focal deficit, seizures, psychoorganic syndrome, and headaches.
Related syndromes
Neurofibromatosis type 2 (NF2)
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Complete surgical resection. Gamma knife radiosurgery or high dose
conformal radiation are options for smaller lesions.
Hydroxyurea for unresectable or recurrent tumors
Notes
Usually fatal, with median survivals of less than 2 years.
Metastatic deposits in lung, pleura, bone, liver can occur.
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Meningioma, angiomatous
9534/0
Meningeal Tumors
Preferred Term
Angiomatous meningioma
Synonyms
None in ICD-O-3
Related terms
Note: this tumor is not the same as angioblastic meningioma, a former name
for hemangiopericytoma. (9150/_)
Definition
Variant of meningioma, a slowly growing, benign tumor attached to the dura
mater and composed of neoplastic meningothelial (arachnoidal) cells with
numerous blood vessels.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9534/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Memingiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Meningiomas in general: neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Differential diagnoses for angiomatous meningioma include vascular
malformations and capillary hemangioblastoma.
Angiomatous meningiomas do not display aggressive clinical behavior.
Meningiomas in general: approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
Meningioma, chordoid
9538/1
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55
Preferred Term
Chordoid meningioma
Synonyms
None in ICD-O-3
Related terms
Clear cell meningioma
Definition
A somewhat aggressive meningioma variant attached to the dura mater and
composed of neoplastic meningothelial (arachnoidal) cells and resembling
chordoma.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9538/1
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Meningiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Chordoid meningiomas ahve been associated with Castleman’s disease.
Meningiomas in general: neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection. High rate of recurrence following subtotal resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Meningiomas in general: approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
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Meningioma, fibrous
9532/0
Meningeal Tumors
Preferred Term
Fibrous meningioma
Synonyms
Fibroblastic meningioma
Related terms
None
Definition
Common variant of meningioma, a slowly growing, benign tumor attached to
the dura mater and composed of neoplastic spindle-shaped cells that appear to
be fibroblastic.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9532/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Meningiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Meningiomas in general: neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Meningiomas in general are approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
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Meningioma, meningothelial
9531/0
Meningeal Tumors
Preferred Term
Meningothelial meningioma
Synonyms
Endotheliomatous meningioma
Syncytial meningioma
Related terms
None
Definition
Classic, common variant of meningioma, a slowly growing, benign tumor
attached to the dura mater in which the tumor cells form lobules like normal
resembling arachnoid tissue.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9531/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Meningiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Meningiomas in general: neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Meningiomas in general are approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
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Meningioma, papillary
9538/3
Meningeal Tumors
Preferred Term
Papillary meningioma
Synonyms
None in ICD-O-3
Related terms
Rhabdoid meningioma (see separate table)
Definition
Rare meningioma variant defined by perivascular pseudopapillary pattern in
at least part of the tumor.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9538/3
ICD-O-3 Grade
9
WHO grade
III
Sites affected
Meningiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Meningiomas in general: neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Papillary meningioma tends to occur in children.
75% have local invasion of the brain and about 55% recur.
Meningiomas in general: approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
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Meningioma, psammomatous
9533/0
Meningeal Tumors
Preferred Term
Psammomatous meningioma
Synonyms
None in ICD-O-3
Related terms
None
Definition
A variant of meningioma, a slowly growing, benign tumor attached to the
dura mater and composed of neoplastic meningothelial cells with abundant
psammoma bodies.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9533/0
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Psammomatous meningioma: thoracic spinal cord.
Meningiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Meningiomas in general :neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Meningiomas in general: approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
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Meningioma, rhabdoid
9538/3
Meningeal Tumors
Preferred Term
Rhabdoid meningioma
Synonyms
None in ICD-O-3
Related terms
Papillary meningioma (see separate table)
Definition
Aggressive uncommon meningioma variant containing neoplastic
meningothelial cells resembling rhabdoid tumors of other sites.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
192.1
C70._
Benign
225.2
D32._
Borderline
237.6
D42._
ICD-O-3 Morphology
9538/3
ICD-O-3 Grade
9
WHO grade
III
Sites affected
Meningiomas in general–Intracranial: Parasagittal cerebral convexities, falx
cerebri, sphenoid wings, olfactory grooves, parasellar region, orbital cavity,
cerebellopontine angle.
Intraspinal: thoracic intradural compartment
Clinical features/
Symptoms
Compression of adjacent structures causing symptoms based on the location
of the tumor. Headache; seizure, motor and sensory symptoms.
Related syndromes
Meningiomas in general: neurofibromatosis type 2; also possible links to
Cowden syndrome and Gorlin syndrome
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Surgical resection.
Radiosurgery may be treatment of choice for unresectable cranial base
meningiomas. Radiation therapy may reduce recurrence rate and prolong
time to recurrence.
Hormonal therapy, hydroxyurea, interferon-a, and the antiprogesterone agent
mifepristone under investigation.
Notes
Rhabdoid meningioma has an aggressive clinical course.
Meningioma in general: approximately 15% of primary intracranial
neoplasms and 25% of primary intraspinal neoplasms. Peak incidence ages
50-69. More common in women. Meningiomas in children tend to be
aggressive.
Can be induced by radiation of varying doses.
Often compress adjacent brain, but rarely invade brain; sometimes invade
adjacent skull. Can encase cerebral arteries but rarely infiltrate artery walls.
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Oligodendroglioma
9450/3
Neuroepithelial–Oligodendroglial Tumors
Preferred Term
Oligodendroglioma, NOS
Synonyms
none in ICD-O-3
Related terms
Well-differentiated oligodendroglioma* (compare to anaplastic
oligodendroglioma--9451/3); WHO grade II oligodendroglioma*; LGO*;
Low-grade oligodendroglioma*
* not listed in ICD-O-3
Definition
Well-differentiated diffusely infiltrating tumor of adults composed
predominantly of cells resembling oligodendrocytes; may have marked
nuclear atypia and rare mitoses, but extensive mitoses, microvascular
proliferation or necrosis meets the criteria for anaplastic oligodendroglioma
(9451/3).
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9450/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Supratentorial cortex and white matter (frontal lobe 50-65%, then temporal,
parietal, occipital in descending order); rarely in cerebellum, brain stem,
spinal cord, leptomeninges.
Clinical features/
Symptoms
Epileptic seizures and headaches (usually a long clinical history).
Related syndromes
Very scanty evidence of association with any hereditary cancer syndrome
Standard treatment
Likely to have:
9 Radiation therapy
: Systemic therapy
Surgical resection; radiation therapy may be recommended for some patients
(usually incompletely resected or age >45). Chemotherapy is under clinical
evaluation for progressive tumors.
Chemotherapy (PVC, melphalan, thiotepa, temozolomide) recommended for
patients with subtotal resection, or for anaplastic or otherwise aggressive
tumors. Observation before chemotherapy for patients with stable, nonenhancing disease.
Notes
Reported as 5-18% of all intracranial tumors.
Peak incidence 40-59 years of age. Mean age in one series was 42.6 years.
Also develops in children: mean age 10 for supratentorial tumors; 7.5 for
infratentorial tumors.
Median survival time 3-5 years; 4-10 years in some studies
Oligodendrogliomas tend to malignant progression less frequently than
astrocytomas.
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Oligodendroglioma, anaplastic
9451/3
Neuroepithelial–Oligodendroglial Tumors
Preferred Term
Anaplastic oligodendroglioma
Synonyms
none in ICD-O-3
Related terms
WHO grade III oligodendroglioma*
* not listed in ICD-O-3
Definition
Diffusely infiltrating tumor of adults composed of cells resembling
oligodendrocytes with extensive mitoses, microvascular proliferation or
necrosis; poorer prognosis than oligodendroglioma (9450/3).
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9451/3
ICD-O-3 Grade
4
WHO grade
III
Sites affected
Supratentorial cortex and white matter (frontal lobe 60%, then temporal,
parietal, occipital in descending order); rarely in cerebellum, brain stem,
spinal cord, leptomeninges. May be multifocal.
Clinical features/
Symptoms
Seizures (similar to anaplastic astrocytoma); problems with speech, vision,
sensation, motor activity; personality/behavior changes. The type of
symptoms may help identify the location of the tumor. Generally a short
clinical history. Symptoms of long duration may indicate malignant
progression from a pre-existing lower grade tumor.
Related syndromes
Very scanty evidence of association with any hereditary cancer syndrome
Standard treatment
Likely to have:
: Radiation therapy
: Systemic therapy
Resective surgery plus external beam radiation therapy. Consider
incompletely resected/unresectable/anaplastic/aggressive tumors for PCV
(procarbazine, CCNU and vincristine), melphalan, thiotepa, temozolomide.
Observation before chemotherapy for patients with stable, non-enhancing
disease. Clinical trial with interstitial brachytherapy, radiosensitizers,
hyperthermia, or intraoperative radiation therapy
Notes
Anaplastic oligodendroglioma represents about 20% of all
oligodendrogliomas.
Mean age at diagnosis 48.7 years (older than grade II oligodendroglioma)
Median survival time: 3.9 years.
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Pineoblastoma
9362/3
Pineal parenchymal Tumors
Preferred Term
Pineoblastoma
Synonyms
Mixed pineal tumor
Mixed pineocytoma-pineoblastoma
Pineal parenchymal tumor of intermediate differentiation (PPTID)
Transitional pineal tumor
Related terms
PB
Pineal parenchymal tumor (PPT)
PNET of the pineal gland is called pineoblastoma.
Definition
A highly malignant, primitive embryonal tumor of the pineal gland with
preferential manifestation in children.
Undifferentiated neuroectodermal tumor or poorly differentiated small round
cell pineal tumor.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
194.4
C75.3
Benign
227.4
D35.4
Borderline
237.1
D44.5
ICD-O-3 Morphology
9362/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Pineal gland (C75.3)
Clinical features/
Symptoms
Hydrocephalus, symptoms of increased intracranial pressure, problems with
eye movement. Hormonal disturbances in children.
Related syndromes
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Radiation or combined chemoradiation. Complete resection is rarely
possible.
Notes
Rare, especially in adults. Usually occur before age 20. Occurrence slightly
higher in males. Pineal tumors account for 0.5%-2% of childhood CNS
tumors.
So-called “trilateral retinoblastoma” includes pineoblastoma and bilateral
retinoblastoma.
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Pineocytoma
9361/1
Pineal parenchymal Tumors
Preferred Term
Pineocytoma
Synonyms
None listed
Related terms
PC
Pineal parenchymal tumor (PPT)
Pinealcytoma
Pinealoma
Definition
Histologically benign, slow growing tumor in the pineal body.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
194.4
C75.3
Benign
227.4
D35.4
Borderline
237.1
D44.5
ICD-O-3 Morphology
9361/1
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Pineal gland (C75.3)
Clinical features/
Symptoms
Increased intracranial pressure, vision changes, changes in mental status,
dysfunction of the brain stem and/or cerebellum, endocrine abnormalities.
Related syndromes
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Complete excision. Subtotal resection followed by postoperative
radiotherapy to a dose of 50 to 55 Gy.
Notes
Rare, especially in adults, less than 1% of all intracranial neoplasms. Pineal
tumors account for 0.5%-2% of childhood CNS tumors. Tend to affect young
adults, but can occur at any age.
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Pituitary adenoma
various codes
Sellar Region Tumors–Endocrine Neoplasms
Preferred Term
Pituitary adenoma
Types of Adenomas
(not synonyms)
8140/0
8140/1
8146/0
8260/0
8270/0
8271/0
8272/0
8280/0
8281/0
8290/0
8300/0
8310/0
8323/0
8333/0
8334/0
Definition
Benign, slowly growing tumor of epithelial origin arising from anterior lobe
(adenohypophysis) of pituitary gland
Adenoma NOS
Atypical adenoma
Monomorphic adenoma
Papillary adenoma, NOS; Glandular papilloma
Chromophobe adenoma (null cell adenoma)
Prolactinoma
Pituitary adenoma, NOS
Acidophil adenoma; Eosinophil adenoma
Mixed acidophil-basophil adenoma
Oxyphilic adenoma; Oncocytic adenoma; Oncocytoma
Basophil adenoma; Mucoid cell adenoma
Clear cell adenoma
Mixed cell adenoma
Microfollicular adenoma; Fetal adenoma
Macrofollicular adenoma; Colloid adenoma
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
194.3
C75.1
Benign
227.3
D35.2
Borderline
237.0
D44.3
ICD-O-3 Morphology
8272/0
ICD-O-3 Grade
9
WHO grade
No WHO grade
Sites affected
C75.1 Pituitary gland
Clinical features/
Symptoms
Headache, vision changes, abnormal hormone production; pressure on
surrounding brain tissue; symptoms (gigantism, galactorrhea, Cushing’s
disease, and others) depend on what abnormal hormones are produced
Related syndromes
Multiple endocrine neoplasia; a small minority of cases associated with
MEN1
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Trans-sphenoidal resection followed by radiation; stereotactic radiosurgery;
gamma knife; however, postoperative radiation is unnecessary after complete
removal. Some types of pituitary adenoma are controllable with exogenous
hormones; dopamine agonists, somatostatin analogues and GH antagonists.
Resection by either sublabial route or newer endonasal route.
Notes
Symptomatic pituitary adenomas are 12-15% of all intracranial tumors; rarely
metastasize. Most commonly discovered in early adulthood; affects males
and females equally
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Pituitary carcinoma
8272/3
Sellar Region Tumors–Endocrine Neoplasms
Preferred Term
Pituitary carcinoma
Synonyms
Pituitary adenocarcinoma, invasive macroadenoma
Definition
Aggressive tumor of epithelial origin arising from anterior lobe
(adenohypophysis) of pituitary gland
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
194.3
C75.1
Benign
227.3
D35.2
Borderline
237.0
D44.3
ICD-O-3 Morphology
8272/3
ICD-O-3 Grade
9
WHO grade
No WHO grade
Sites affected
C75.1 Pituitary gland
Clinical features/
Symptoms
Vision changes, abnormal hormone production; pressure on surrounding
brain tissue; symptoms (gigantism, galactorrhea, Cushing’s disease, and
others) depend on what abnormal hormones are produced
Related syndromes
Standard treatment
Likely to have:
: Radiation therapy
9 Systemic therapy
Trans-sphenoidal resection followed by radiation; stereotactic radiosurgery;
gamma knife; some types of pituitary tumors are controllable with exogenous
hormones, dopamine agonists, somatostatin analougues and GH antagonists.
Resection by either sublabial route or newer endonasal route.
Notes
Very rare. Most pituitary carcinomas arise from previously operated or
irradiated invasive adenomas. Like adenomas, they are classified by the
presumable cell of origin and the hormone produced.
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Primitive neuroectodermal tumor
9473/3
Embryonal Tumors
Preferred Term
Primitive neuroectodermal tumor, NOS
Synonyms
PNET, NOS
Central primitive neuroectodermal tumor, NOS
CPNET
Supratentorial PNET (SPNET)
Related terms
Cerebral neuroblastoma
Central neuroblastoma
Definition
An embryonal tumor in the cerebrum or suprasellar region composed of
undifferentiated or poorly differentiated neuroepithelial cells.
Malignant tumor arising from cells that are believed to remain from fetal
brain development.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9473/3
ICD-O-3 Grade
9
WHO grade
IV
Sites affected
Cortex, cerebrum or suprasellar.
PNET in or near a spinal fluid sac known as the fourth ventricle is called
medulloblastoma (see separate table). PNET of the pineal gland is called
pineoblastoma (see separate table).
Clinical features/
Symptoms
Cerebrum: Seizures, disturbances of consciousness, increased intracranial
pressure or motor deficit.
Suprasellar: Vision changes, endocrine problems. Increased head
circumference in infants.
Related syndromes
Standard treatment
Likely to have:
: Radiation therapy
: Systemic therapy
Surgery. Adults: Radiation and chemotherapy. Young children:
chemotherapy alone.
Notes
Usually occur in early childhood but may become symptomatic in adult life.
The usual age range is 4 weeks to 10 years. Males are twice as likely to be
affected compared to females.
PNETs have a tendency to spread over the brain and spinal cord by way of
the spinal fluid.
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Subependymoma
9383/1
Neuroepithelial-Ependymal Tumors
Preferred Term
Subependymoma
Synonyms
Subependymal glioma
Subependymal astrocytoma
Related terms
Mixed subependymoma-ependymoma
Subependymal glomerate astrocytoma* (usage discouraged)
* not listed in ICD-O-3
Definition
Slow growing neoplasm of glial cell clusters usually attached to the wall of a
ventricle. Very low mitotic activity.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191.5, 7; 192.2
C71.5, 7; C72.0
Benign
225.0, 3237.5
D33.0, 1, 4
Borderline
D43.0, 1, 4
ICD-O-3 Morphology
9383/1
ICD-O-3 Grade
9
WHO grade
I
Sites affected
Fourth ventricle (50-60%), then lateral ventricles (30-40%), less commonly
the third ventricle, septum pellucidum, and spinal cord.
Clinical features/
Symptoms
Ventricular obstruction and increased intracranial pressure.
Asymptomatic subependymomas are occasionally found at autopsy.
Related syndromes
None known
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Surgical resection is usually curative. Radiation may be considered
depending on the amount of residual tumor.
In children, chemotherapy may be considered in place of radiation after
surgical resection.
Notes
All age groups are susceptible, but most common in middle-aged and elderly
males. Often asymptomatic and found only at autopsy.
Subependymoma generally has a good prognosis.
Mixed ependymoma-subependymoma has a clinical course similar to
ependymoma with WHO grade II.
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Xanthoastrocytoma, pleomorphic
9424/3
Neuroepithelial–Astrocytic Tumors
Preferred Term
Pleomorphic xanthoastrocytoma
Synonyms
None in ICD-O-3
Related terms
PXA*; Pleomorphic xanthoastrocytoma with anaplastic features*
(terminology for PXA with more aggressive characteristics); Xanthomatous
astrocytoma*
* not listed in ICD-O-3
Definition
Recently described, rare variant of astrocytoma arising from subpial
astrocytes with circumscribed growth pattern.
Casefinding
ICD-9-CM
ICD-10-CM
Malignant
191._
C71._
Benign
225._
D33._
Borderline
237.5
D43._
ICD-O-3 Morphology
9424/3
ICD-O-3 Grade
9
WHO grade
II
Sites affected
Supratentorial (98%) in superficial location involving cerebrum and
meninges (meningo-cerebral); temporal lobe is most frequent, then frontal
and parietal. Rare in cerebellum and spinal cord. Case reports of retina as
primary site.
Clinical features/
Symptoms
Seizures (often a long history).
Related syndromes
No specific association with hereditary tumor syndromes; rarely linked with
neurofibromatosis type 1 (NF-1).
Standard treatment
Likely to have:
9 Radiation therapy
9 Systemic therapy
Complete surgical removal, if possible. For incompletely resected tumors,
the use of radiation in young patients must be weighed against its long-term
sequelae.
No role for adjuvant radiotherapy or chemotherapy.
Notes
< 1% of all astrocytic neoplasms; only about 200 cases in world literature
Most common in 10-19 age range.
Generally favorable prognosis. Slow evolution with long symptom-free
periods. Overall survival at 10 years: 70%
May not infiltrate, but may spread along meninges.
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SYNDROMES ASSOCIATED WITH CENTRAL NERVOUS SYSTEM TUMORS
Syndromes involving neurofibromas
Neurofibromatosis I (NF-1, Recklinghausen’s disease, von Recklinghausen’s disease, peripheral
neurofibromatosis) hereditary syndrome characterized by multiple cafe-au-lait spots and neurofibromas
on or under the skin. Enlargement and deformation of bones and curvature of the spine (scoliosis) may
also occur. Occasionally, tumors may develop in the brain, on cranial nerves, or on the spinal cord.
Neurofibromatosis II (NF-2, bilateral acoustic neurofibromatosis) genetic disorder in which benign
tumors develop on auditory nerves
Syndromes involving the retina and optic nerve
Familial retinoblastoma autosomal dominant syndrome characterized by early onset (birth through
about 7 years) and bilaterality of retinal malignancies, associated with a long-term predisposition to other
types of cancer
Von Hippel-Lindau disease (familial cerebello-retinal angiomatosis, Lindau’s disease, cerebroretinal
angiomatosis) congenital hemangiomatosis of the retina and cerebellum
Syndromes involving endocrine glands
Multiple endocrine neoplasia type I (MEN type 1, Wermer syndrome, multiple endocrine adenomatosis
type 1, MEA type 1) polyendocrine neoplasia
Multiple endocrine neoplasia type II (MEN type 2, Sipple syndrome, familial chromaffinomatosis,
multiple endocrine adenomatosis type 2, MEA type 2, PTC {pheochromocytoma-thyroid carcinoma}
syndrome) hereditary association of pheochromocytoma with medullary thyroid carcinoma
Syndromes involving neoplasms of other body systems
Gorlin syndrome (Gorlin-Goltz syndrome, Hermans-Grosfeld-Spaas-Valk disease, nevoid basal cell
carcinoma syndrome, basal cell carcinoma syndrome, basal cell nevus syndrome) inherited predisposition
to develop multiple basal cell carcinomas and multiple cysts within the jaws; multiple nevoid basal cell
epithelioma, jaw cysts and bifid ribs
Li-Fraumeni syndrome (LFS) a rare autosomal dominant syndrome in which patients are predisposed to
cancer a wide variety of cancers beginning at a young age and the potential for multiple primary sites of
cancer during the lifetime of affected individuals, particularly early breast cancer, soft tissue sarcomas,
bone sarcomas, adrenal cortical carcinoma, acute leukemia, and brain tumors
Turcot syndrome (adenomatous polyposis syndrome) hereditary syndrome marked by development of
malignant brain tumors (gliomas) and multiple polyps of the colon (adenomatous polyposis coli)
Cowden Disease (multiple hamartoma syndrome, Cowden syndrome) familial syndrome involving
abnormalities of the central nervous system and defects of many body structures, including hypertrophy
of the breasts with fibrocystic disease and early malignant degeneration; causes hamartomatous
neoplasms of the skin and mucosa, GI tract, bones, central nervous system (CNS), eyes, and genitourinary
tract, and associated with increased incidence of breast and thyroid malignancies.
Tuberous sclerosis (Bourneville-Pringle syndrome, Bourneville’s disease, adenoma sebaceum syndrome,
epiloia, tuberous sclerosis complex) genetic disorder that causes benign tumors to form in many different
organs, primarily in the brain, eyes, heart, kidney, skin and lungs.
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WHO Grade for CNS Tumors
The WHO system assigns a numerical grade representing the overall biologic potential, on an ascending
scale of malignancy of I (benign) to IV (malignant). There are many well accepted histologic grading
systems for CNS tumors; therefore, this system should be specifically identified when used (e.g. WHO
Grade I). Not all CNS histologies are graded in the WHO system.
The following general definitions of WHO grade are applied to the tissue by the pathologist:
GRADE I (e.g. pilocytic astrocytoma).
Tumours with a low proliferative potential, a frequently discrete nature, and a possibility of cure
following surgical resection alone.
GRADE II
Generally infiltrating tumours low in mitotic activity, but with a potential to recur. Some tumour
types tend to progress to lesions with higher grades of malignancy (e.g. well-differentiated
astrocytomas, oligodendrogliomas and ependymomas).
GRADE III
Histological evidence of malignancy, generally in the form of mitotic activity, clearly expressed
infiltrative capabilities, and anaplasia.
GRADE IV
Mitotically-active, necrosis-prone neoplasms, generally associated with a rapid pre- and postoperative
evolution of the disease.
Source: European Network of Cancer Registries, http://www.encr.com.fr/workgr1D.htm
Comparison of Grading Systems for Astrocytic Tumors
WHO designation
WHO Kernohan St. Anne/ St. Anne/Mayo criteria
grade* grade*
Mayo grade
pilocytic astrocytoma
I
I
excluded
-
astrocytoma
II
I, II
1
no criteria fulfilled
2
one criterion: usually nuclear atypia
anaplastic (malignant)
astrocytoma
III
II, III
3
two criteria: usually nuclear atypia and
mitosis
glioblastoma
IV
III, IV
4
three or four criteria: usually the above and
necrosis and/or endothelial proliferation
*The WHO and Kernohan systems are not criteria based. Thus, a given tumor may not fall under the same
designation in all three systems.
Source: http://www.bgsm.edu/bgsm/surg-sci/ns/newwhobt.html
Kernohan Classification of Astrocytomas (1949)
Grade 1: increased cellularity of astrocytes
Grade 2: mild –– mod nuclear polymorphism, no mitotic figures
Grade 3: 50-75% astrocytes are normal, frequent mitotic figures, increased vascularity, necrosis.
Grade 4: marked cellular pleomorphism, extensive endothelial proliferation, numerous mitotic figures,
necrosis
Source: http://www.angelfire.com/retro/michaelpoon168/astrocytoma.htm
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INDEX BY ICD-O-3 MORPHOLOGY CODE
8272/0
8272/3
9350/1
9361/1
9362/3
9381/3
9382/3
9382/3
9383/1
9384/1
9390/3
9390/0
9391/3
9391/3
9391/3
9391/3
9392/3
9392/3
9393/3
9394/1
9400/3
9401/3
9410/3
9411/3
9412/1
9413/0
9420/3
9421/1
9424/3
Pituitary adenoma 65
Pituitary carcinoma 66
Craniopharyngioma (Rathke’s pouch tumor)
33
Pineocytoma 65
Pineoblastoma; Pineal parenchymal tumor
of intermediate differentiation 64
Gliomatosis cerebri 50
Anaplastic oligoastrocytoma 49
Mixed glioma 49
Subependymoma 69
Subependymal giant cell astrocytoma 30
Choroid plexus carcinoma 31
Choroid plexus papilloma NOS 32
Ependymoma NOS 37
Clear cell ependymoma 37
Tanycytic ependymoma 37
Cellular ependymoma 37
Anaplastic ependymoma 38
Ependymoblastoma 36
Papillary ependymoma 40
Myxopapillary ependymoma 39
Astrocytoma, NOS 23
Anaplastic astrocytoma 24
Protoplasmic astrocytoma 29
Gemistocytic astrocytoma 27
Desmoplastic infantile astrocytoma;
Desmoplastic infantile ganglioglioma 25
Dysembryoplastic neuroepithelial tumor 34
Fibrillary astrocytoma 26
Pilocytic astrocytoma 27
Pleomorphic xanthoastrocytoma 70
SEER Program Training Materials
Draft #2 September 2003
9430/3
9440/3
9441/3
9442/3
9442/1
9444/1
9450/3
9451/3
9470/3
9473/3
9492/0
9493/0
9505/3
9505/1
9530/0
9530/0
9530/0
9530/0
9530/0
9530/3
9531/0
9532/0
9533/0
9534/0
9538/1
9538/3
9538/3
Astroblastoma 22
Glioblastoma, NOS 44
Giant cell glioblastoma 46
Gliosarcoma 50
Gliofibroma 47 (not in WHO
classification)
Chordoid glioma 48
Oligodendroglioma, NOS 62
Oligodendroglioma, anaplastic 63
Medulloblastoma NOS; Melanotic
medulloblastoma 52
Primitive neuroectodermal tumor, NOS
[Supratentorial PNET] 68
Gangliocytoma 41
Dysplastic gangliocytoma of cerebellum
(Lhermitte-Duclos) 35
Ganglioglioma, anaplastic 43
Ganglioglioma, NOS 42
Secretory meningioma NOS 53
Lymphoplasmacyte-rich meningioma
NOS 53
Microcystic meningioma NOS 53
Meningioma NOS 53
Metaplastic meningioma NOS 53
Malignant meningioma [Anaplastic] 54
Meningothelial meningioma 58
Fibrous meningioma 57
Psammomatous meningioma 60
Angiomatous meningioma 55
Chordoid meningioma 56
Papillary meningioma 59
Rhabdoid meningioma 61
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INDEX OF ALL TERMS...
...
...
...
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