Cancer In Children
UPCON – Cancer Day For Primary Care
Geoff Cuvelier MD FRCPC
Pediatric Oncologist
CancerCare Manitoba
January 31, 2014
Disclosures
• None
• I will not be discussing any off-label indications for any drugs
or medical devices.
• I’m really sympathetic towards family doctors and their role in
diagnosing children with cancer.
Objectives
1. To increase understanding among family physicians and
other front-line healthcare workers that cancer in children is
a rare, but identifiable problem.
1. To discuss common presentations of acute leukemia and
brain tumors in children.
2. To impart that childhood cancer can be a great mimicker of
other more common pediatric problems.
Types of Childhood Cancer
• * 1. Leukemia (ALL>AML>Chronic Leukemia) ……….31.0%
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* 2. CNS tumors………………………………………… 18.3%
* 3. Lymphomas (Hodgkin’s, NHL) ……………………13.8%
4. Neuroblastoma ……………………………………….. 6.8%
5. Soft tissue sarcomas …………………………………..6.2%
6. Wilms’ tumors ………………………………………. 5.7%
7. Bone tumors ………………………………………….. 4.7%
8. Retinoblastoma ………………………………………. 2.5%
9. Germ cell tumors …………………………………….. 2.4%
10. Liver tumors …………………………………………… 1.3%
11. Other …………………………………………………… 7.4%
How Often Will Family Doctors See A Case of
Childhood Cancer?
• Infrequently:
• Children (<15 yrs):
• Adults:
14.7 cases / 100,000 children / year.
467.7 cases / 100,000 adults / year
• Manitoba: About 60 new cases childhood cancer / year
• In primary care …. Perhaps one child every 10-15 years.
• Problem: The parent expects you will know what to do and they are
not happy when the diagnosis has been missed! (may have multiple
health care visits)
So…Isn’t Pediatric Oncology Sad?
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Well sometimes … but not usually. In very “general” terms - Long-Term Survival:
1962
2013
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Acute Lymphoblastic Leukemia
4%
93%
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Acute Myelogenous Leukemia
4%
60% (variable on genetics)
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CNS tumors
35%
74% (variable on type)
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Hodgkin’s Disease
50%
95%
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NHL
6%
84%
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Neuroblastoma
10%
69% (variable on stage)
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Wilms’ tumor
50%
92%
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Osteosarcoma
20%
63%
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Ewing’s sarcoma
5%
65% (variable on stage)
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Rhabdomyosarcoma
30%
64% (variable on stage)
Why Cancer Diagnosis Is Missed in Children
• Not expecting the diagnosis “Cancer Doesn’t Happen in Children”
• The parents are telling the story – quick assumptions
• Mimics other conditions
– “Mono” – Acute Leukemia, Lymphoma
– “Asthma” – Acute Leukemia with Mediastinal Mass
– “Tension Headache” / Migraine – Brain Tumor
– “Trauma” – Osteogenic sarcoma
Case One
• 3 yr old girl brought to walk-in-clinic
• Pale and tired for 1 week, fevers
• Dx: “Viral Infection”
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1 week later:
Still tired, pale, fevers
Limping, “Legs hurt”
Dx: “Growing Pains”
Case One
• 1 week later:
• Getting worse
• Now getting a red rash
Petechiae – Sign of
Thrombocytopenia
Case One
• Physical Exam:
• HR: 120 BP: 100/70 RR: 34 Temp: 38.8oC
• Unwell, Tired Appearing
• Subconjunctival hemorrhages
• Cervical Lymphadenopathy
• Hyperdynamic precordium
• Systolic flow murmur
• Hepatosplenomegaly to just
above umbilicus.
** What is the most important test?
Complete Blood Count
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WBC:
Hemoglobin:
Platelets:
Neutrophils:
Lymphocytes:
Other Cells:
35 x 109/L (N: 5-15)
45 g/L (N: 120-160)
2 x 109/L (N: 150-450)
0.2 x 109/L (N: 2.5-7.0)
1.0 x 109/L (N: 2.0-7.0)
33.5 x 109/L
(N: none)
• What is the most likely diagnosis?
• Acute Leukemia
Blood Smear Under Microscope
Normal
Patient – Acute Leukemia
“Other Cells” = Blasts
“Acute Lymphoblastic Leukemia”
Acute Leukemia Presentation
• Often over short time – days to weeks.
• Constitutional symptoms:
– Fevers
– Malaise (often not going to school)
– Anorexia / weight loss (sometimes)
• Consequence of bone marrow being packed with blasts:
– Limping
– Bone Pain (legs, pelvis especially)
– Hematologic (anemia / thrombocytopenia)
Acute Leukemia Presentation
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Consequence of anemia:
– Pallor
– Tired / Headaches
– Tachycardia / Tachypnea
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Consequence of thrombocytopenia:
– Petechiae / Purpura
– Subconjunctival Hemorrhages
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Consequence of organ infiltration:
– Hepatosplenomegaly
– Lymphadenopathy
– Mediastinal mass
– Testiculomegaly
Case Two - Variation on the Leukemia Theme
• 14 year old boy presents with one week fevers, bone pain,
headaches, tired and pale. Now trouble breathing.
• O/E: Subconjunctival hemorrhages, multiple petechiae,
lymphadenopathy, hepatosplenomegaly and facial plethora and
fullness.
• CBC:
WBC: 252 x 109/L 98% Blasts
(=Hyperleukocytosis)
Hg: 75 g/L Plts: 2 x 109/L
Mediastinal Mass and SVC Syndrome with
Hyperleukocytosis = Emergency
• Mediastinal Mass:
– Compromised airway
– Anesthesia concerns
• SVC Obstruction
– Facial, Neck, and Arm
Edema
– Poor venous return from
CNS – potential for
stroke and cerebral
edema.
Adolescent with hyperleukocytosis and anterior mediastinal mass likely T-cell ALL.
The Anterior Mediastinal Mass
• Wide differential diagnosis – some malignant some not.
• “Terrible T’s”:
– T-cell ALL
– T-lymphoblastic lymphoma (cousin disorder to T-ALL)
– Thymoma (or normal thymus in infant)
– Thyroid Cancer
– Teratoma (germ cell tumor)
– Tuberculosis
• Hodgkin’s Lymphoma
Normal Thymus of Infancy
Wavy Sign
Looks like a mediastinal mass – don’t be confused. Most infants <1 years
old you will see this and it is normal. Involutes between 1-3 years. Only
2% will still be visible on CXR by 4 years.
Case Three
• 4 year old, bit more cranky and irritable over last few
weeks. Decreased play.
• Complaining of headaches more often past week.
• Mom has migraine history and thought it was this.
Doctor agrees.
• Dx: Migraines. Tylenol, rest, fluids.
• Comes back – vomiting, walking abnormally.
• Sees the family physician who came to this talk and
remembered…….
The Headache RED FLAGS
• A headache is a brain tumor until proven otherwise when:
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Child 7 or less years.
Particularly bad in the early morning.
Awakening at night from headache.
Progressively getting worse over time.
Associated with increased ICP or other neurologic symptoms
and signs – vomiting, ataxia, nystagmus, deteriorating
developmental milestones or school perfomance.
Why Pediatric Brain Tumors are Missed
• Not listening to the patient / parent – history, history , history
• Not remembering the headache red flags.
• Not doing a head circumference on an infant with open fontanelles
and appreciating it is getting bigger too fast.
• Not appreciating declining school performance or behavioral
disturbance with headaches could be a brain tumor.
• You need a CT scan of the head to rule in/out a brain tumor.
Case Four
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14-year old boy. Increasing “swelling”
of neck. 3 Large matted palpable
masses (lymphadenopathy) in R
anterior triangle and a supraclavicular
node.
Fevers, tired, night-sweats, 20 pound
weight loss in last 4 weeks
Confused with infectious
mononucleosis (EBV).
Normal CBC and Differential.
LDH: 1400 (N: 150-350)
Mediastinal Mass
Clinical Pearl
• Small (1x1cm)
lymphadenopathy in
anterior/posterior
cervical triangle or
occipital area is common
in children and often (but
not always) related to
infection…. BUT
• A supraclavicular node
is cancer until proven
otherwise.
Node
Clavicle
Questions?