ORIGINAL
ARTICLE
SUPRACRICOID PARTIAL LARYNGECTOMY AS
A PRIMARY
TREATMENT FOR CARCINOSARCOMA OF THE LARYNX
Supracricoid partial laryngectomy
as a primary treatment for
carcinosarcoma of the larynx
Kuauhyama Luna-Ortiz, MD; Adalberto Mosqueda-Taylor, DDS, MSc
Abstract
Carcinosarcoma of the larynx is uncommon. When it does
occur, its clinical features resemble those of sarcomatoid
carcinoma, and its biologic behavior is similar to that of
malignant mesenchymal neoplasms. We describe 2 cases
of carcinosarcoma of the glottis. The tumors were staged
as T3N0M0 and T2N0M0. Both patients were treated with
supracricoid partial laryngectomy with cricohyoidoepiglottopexy.Eightmonthspostoperatively,1ofthe2patients
experiencedarecurrenceofthesarcomatouscomponentof
thetumor,andheunderwentatotallaryngectomy.Theother
patient remained free of disease at 12 months of follow-up.
We conclude that supracricoid partial laryngectomy may
beofferedasanorgan-preserving measure even in patients
with sarcomatous disease; total laryngectomy can be held
in reserve as a rescue measure. Patients must be closely
monitored for early detection of recurrence. The role of
adjuvant therapy for sarcomatous neoplasms in this area
has not yet been clearly established.
Introduction
Carcinosarcoma of the head and neck is uncommon. Most
reported cases have occurred in the major salivary glands;
other sites include the larynx and pharynx and, less frequently, the oral and nasal cavities and the esophagus.1-6
True carcinosarcomas of the larynx and the hypopharynx
represent less than 1% of all malignant neoplasms in this
area.3,4,6-8 The mean age of carcinosarcoma patients is 60
years, and there is a male predominance (3.5 to 1).4,6-8 Risk
factors are similar to those of squamous cell carcinoma
From the Department of Head and Neck Surgery, Instituto Nacional de
Cancerología, Tlalpan (Dr. Luna-Ortiz), and the Oral Pathology
Laboratory, Universidad Autónoma Metropolitana Xochimilco,
Col. Villa Quietud (Dr. Mosqueda-Taylor), México.
Reprint requests: Kuauhyama Luna-Ortiz, MD, Departamento de Cabeza
y Cuello, Av. San Fernando #22, Tlalpan D.F. 14080, México,
Phone: 52-55-5628-0400, ext. 285; fax: 52-55-5628-0435; e-mail:
[email protected] or [email protected]
Originally presented at the Sixth International Conference on Head and
Neck Cancer; Aug. 11, 2004; Washington, D.C.
Volume 85, Number 5
(e.g., smoking and alcoholism), and there is a higher risk in
patients who are exposed to radiation. The classic clinical
presentationfeaturesdysphoniaanddyspneasecondaryto
laryngeal obstruction.5-7,9
The histogenesis of carcinosarcoma is controversial.
The most widely accepted theory ascribes its origin to the
differentiation of primitive blastic mesenchymal cells that
can mature and produce malignant neoplasms of multiple
differentiation.Atdiagnosis,atruecarcinosarcomaexhibits
amalignantmesenchymalcomponent(sarcoma),aswellas
an epithelial component (carcinoma or adenocarcinoma).
Histologic study will reveal the presence of pleomorphic
cells and atypical mitosis.1-4,7 In cases when histologic
lineages that are uncommon in the larynx have been found,
treatment has almost invariably involved total laryngectomy. Only a few reports exist of cases of nonsquamous
(nonepidermoid) histology in which organ preservation
was attempted.6,10 To our knowledge, no case of laryngeal
carcinosarcoma treated with supracricoid partial laryngectomy and cricohyoidoepiglottopexy has been previously
reported. In this article, we describe 2 such cases, and we
discuss their salient clinical and histologic features.
Case reports
Patient 1. A 55-year-old man sought medical attention for a
4-monthhistoryofprogressivedysphoniaandrecent-onset
dyspnea. He had been a heavy smoker and heavy drinker
since the age of 15 years. Another physician had previously performed a biopsy of the larynx, which revealed
the presence of a carcinosarcoma.
Our clinical and endoscopic examination revealed that a
tumorhadarisenfromthelefttruevocalfoldandobstructed
90% of the endolaryngeal lumen. The affected vocal fold
was fixed, and the arytenoids were mobile. The tumor was
staged as T3N0M0.
The patient underwent a supracricoid partial laryngectomy with cricohyoidoepiglottopexy, which preserved the
arytenoids, and a selective bilateral dissection from levels
II through V, which preserved both jugular veins and the
XIth cranial nerve. Transoperative and definitive studies
337
LUNA-ORTIZ, MOSQUEDA-TAYLOR
confirmed the diagnosis of carcinosarcoma and revealed
thatthesurgicalmarginswerelesion-free.Nocomplications
occurredduringthepostoperativeperiod.Onpostoperative
day 6, the tracheotomy tube was removed and the patient
was able to speak. He was discharged the following day
with a nasogastric feeding catheter in place. The catheter
was removed on postoperative day 21. The patient was
not considered for postoperative radiotherapy.
Eight months later, the patient reported respiratory
difficulties and odynophagia. Physical exploration and
endoscopy detected a 4-cm tumor in the neoglottic area at
the site of the left arytenoids.The patient underwent a total
laryngectomy. He was monitored with periodic physical
examinations, fiberoptic nasolaryngoscopy, chest x-rays,
and computed tomography (CT) of the head and neck. At
the 12-month examination, he exhibited no evidence of
tumoral activity.
Macroscopic evaluation. The specimen excised during
the first surgery included thyroid cartilage and newly
formedred-violetendopharyngealtissue,whichhadarisen
from the left true vocal fold. The mass had a polypoid appearance, a pedunculated base, and small areas of surface
ulceration (figure 1, A). It measured 3 × 2.5 × 2 cm. The
base of the tumor was continuous, with an irregular plaquelike white lesion that extended along the vocal process of
the arytenoids. The right false and true vocal folds were
without alteration. Sagittal sectioning revealed that the
newly formed tissue was solid and featured alternating
white and violaceous areas.
Microscopic evaluation. At the histologic level, the
newly formed tissue contained two types of cells. The
major component was pleomorphic fusiform cells; these
cells had ovoid nuclei and eosinophilic cytoplasm, and
they exhibited numerous mitotic figures (figure 1, B). The
cells were continuous, with areas of osteoid material. In
someareas,epithelialductsthatcontainedcarcinomawere
present within the sarcomatous neoplasm (figure 1, C).
Immunohistochemical study revealed intense immunoreactivity to cytokeratin in the carcinomatous areas and to
vimentin in the sarcomatous areas. The final diagnosis was
carcinosarcoma of the left true vocal fold that extended to
the vocal process of the arytenoids and infiltrated Reinke’s
space and the skeletal muscle. Dissection of both sides of
the neck turned up no evidence of metastasis.The recurrent
tumor was made up of only the sarcomatous component
of carcinosarcoma (osteosarcoma).
Patient 2. A 54-year-old man with progressive dysphonia of 4 months’ evolution and a 32-year history of
smoking 15 cigarettes per day was referred to our hospital
for management of a malignant laryngeal tumor. Another
physician had biopsied the larynx and rendered a diagnosis
of squamous cell carcinoma.
Our physical and endoscopic examinations revealed
338
A
B
C
Figure 1. Patient 1. A: The excised carcinosarcoma has a polypoid
appearance. B: Microscopic analysis reveals malignant osteoid
material and atypical mitosis. C: Slide shows severe epithelial
dysplasia of the duct.
that a 3-cm tumor located on the right true vocal fold had
obstructed approximately 80% of the endolaryngeal lumen. Vocal fold and arytenoid mobility was normal, and
the tumor was staged as T2N0M0.
We performed a supracricoid partial laryngectomy with
cricohyoidoepiglottopexy and bilateral dissection of the
neck from levels II through V. Intraoperative histopathology confirmed that the surgical margins were clear. During
the postoperative period, the patient developed edema of
ENT-Ear, Nose & Throat Journal  May 2006
SUPRACRICOID PARTIAL LARYNGECTOMY AS A PRIMARY TREATMENT FOR CARCINOSARCOMA OF THE LARYNX
A
II dysplasia (figure 2, B). No continuity existed between
the two neoplastic components. Immunohistochemical
study revealed intense immunoreactivity to vimentin
and actin in the sarcomatous areas and to cytokeratin in
the carcinomatous areas. The definitive histopathologic
diagnosis was a carcinosarcoma made up of areas of in
situ carcinoma and leiomyosarcoma of the right true vocal
fold that involved the vocal process of the arytenoid and
infiltrated into Reinke’s space without affecting the vocal
muscle or thyroid cartilage. Dissection of both sides of the
neck revealed no metastasis.
B
Figure 2. Patient 2. A: Area of leiomyosarcoma with abundant
pleomorphic cells and atypical mitosis. B: Intraepithelial neoplasm adjacent to leiomyosarcoma.
the arytenoids, which was alleviated by steroid therapy.
He was decannulated on postoperative day 10 and was
able to speak. He was discharged on postoperative day 11
with a nasogastric catheter. The catheter was removed on
postoperative day 23. Radiotherapy was not considered.
At 12 months of follow-up, no evidence of tumoral activity was observed.
Macroscopic evaluation. The surgical specimen was
made up of newly formed white, multinodular, polypoid,
pedunculated endolaryngeal tissue that was located on
the right true vocal fold. The mass measured 2.7 × 2.1 ×
1.4 cm and contained areas of hemorrhage. The false and
true left vocal folds were devoid of tumor. Sagittal section
identified a solid, firm neoplasm with alternating white and
violaceous areas. Thyroid cartilage was clearly evident at
the periphery of the specimen.
Microscopic evaluation. Again, histology revealed two
cellulartypesand,again,themajorcomponentswereround
andfusiformpleomorphiccellswithabundanteosinophilic
cytoplasm exhibiting a high mitotic index (figure 2, A).
Other areas contained foci of in situ carcinoma and grade
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Discussion
Carcinosarcomaissometimescalledbyseveralothernames
(pseudosarcoma, sarcomatoid carcinoma, pleomorphic
carcinoma, and pseudocarcinosarcoma), not all of which
are correct.6,8,11,12 The variety of terms, as well as the tumor’s
variable clinical presentation and morphologic characteristics, has the potential to cause some confusion.
Carcinosarcoma vs. sarcomatoid carcinoma. There
is a difference between carcinosarcoma and sarcomatoid
carcinoma; the latter is a squamous cell carcinoma with
spindle-cell changes that simulate a sarcoma. Also, a
sarcomatoid carcinoma grows more slowly. Clinically,
dysphonia is present in patients with both neoplasms, but
only carcinosarcoma is generally associated with dysphagia, dyspnea, and obstruction as an initial symptom.
Most carcinosarcomas are longer than 2 cm; sarcomatoid
carcinomas are usually smaller. Regional metastasis of the
epithelial component of carcinosarcoma occurs in only 10
to 20% of cases, but distant metastasis of the mesenchymal
component is more common.1,2 Differences also exist with
respecttothehistogenic,histopathologic,andimmunohistochemical features of the two tumors (table).1,3,4,6-9,11-13
The sarcomatous components of carcinosarcoma are, in
decreasing order of frequency, chondrosarcoma, osteosarcoma,fibrosarcoma,andmalignantfibroushistiocytoma.2-4
Carcinosarcoma has an aggressive biologic behavior, and
it is associated with high rates of recurrence and distant
metastasis despite radical primary treatment.1,3,5,7,9 The
diagnosis is usually established early on the basis of the
clinical presentation, which is generally characterized by
the presence of an exophytic tumor that quickly causes
obstruction as a result of its rapid growth, especially the
growthofthemesenchymalcomponent.Whenulcerationis
present, the affected area can become overinfected, which
can lead to a false-negative biopsy and delay diagnosis.
Therefore, patients with suspected carcinosarcoma should
undergo a deep biopsy of a nonulcerated area.
Treatment of carcinosarcoma. There is no consensus
as to the most appropriate therapy for carcinosarcoma.6
The most common treatment is total laryngectomy,
complemented in some cases by adjuvant chemotherapy
339
LUNA-ORTIZ, MOSQUEDA-TAYLOR
Table. Comparison of histogenesis and the histologic and immunohistochemical features of sarcomatoid
carcinoma and carcinosarcoma
Histogenesis
Sarcomatoid carcinoma Squamous cell carcinoma with an atypical
pseudosarcomatous stromal reaction; epithelial with transformation to fusiform
morphology
Primitive mesenchymal blastic cell differentiation that can mature and
produce malignant neoplasms of
multiple differentiation
Histology
Dysplasia to invasive carcinoma; mesenchymal carcinoma with pleomorphic cell component or fusiform cells, scarce or no mitoses, and bizarre cells; areas of transition are observed
Dysplasia to invasive carcinoma or
adenocarcinoma; true sarcoma is the
major component with high mitotic index
and cell pleomorphism; no areas of
transition are observed
Immunohistochemistry
Epithelial component is positive to cytokeratin; sarcomatoid component is
positive to cytokeratin and occasionally positive to vimentin with coexpression to cytokeratin Epithelial component is positive to cytokeratin; mesenchymal component is positive to vimentin and other markers,
(S-100, actin, desmin, etc.) depending on
the tumor’s origin
and/or radiotherapy because the prognosis is dependent
on the type of malignant mesenchymal component.1-5,13
We agree with Ianniello et al,6 who suggested that the
therapeuticapproachtoandprognosisforcarcinosarcoma
is similar to that of squamous cell carcinoma. Because
conservative surgery has been shown to provide just as
much local control as radical surgery, supracricoid partial
laryngectomy is indicated for selected cases of squamous
cell carcinoma and nonsquamous cell carcinoma.10,14-16
The quality of life and phonation in patients who have
undergone this type of surgical procedure at our institution has been previously reported.17 In our experience,
these patients were able to achieve an almost normal
biopsychosocial integration.
Supracricoid partial laryngectomy has an advantage
over other partial surgeries with respect to surgical margins.10 In 2001, 2 reports of mesenchymal tumors treated
with conservative surgery were published; Ianniello et al6
reported using vertical hemilaryngectomy, and Veivers et
al10 used supracricoid partial laryngectomy with cricohyoidoepiglottopexy.The case reported by Ianniello et al6 was
diagnosed as a carcinosarcoma, and the case from Veivers
et al10 was diagnosed as a leiomyosarcoma; in retrospect,
however, the latter might also have been a carcinosarcoma
because the patient developed metastatic squamous cell
carcinoma from an unknown primary 6 months after the
initial treatment. Because carcinosarcoma is a high-grade
malignant neoplasm that can metastasize, and because
neck dissections are associated with low mortality,1 we
agree with Veivers et al10 that elective neck dissection
340
Carcinosarcoma
must be performed at the time of the initial treatment of a
nonepithelial neoplasm.
Our2patientswerenotconsideredforadjuvanttreatment
withradiotherapybecausetheirtumorswereconfinedtothe
endolarynx and both necks were negative for metastasis.
The recurrence in patient 1 involved an osteosarcoma,
which is not an especially radiosensitive neoplasm; therefore, the patient was rescued by a total laryngectomy. It is
interesting to note that in most cases of nonsquamous cell
cancer of the larynx, the most common cause of death is
a distant (lung) metastasis.7,10
In conclusion, we believe laryngeal carcinosarcoma can
be managed with conservative organ-preserving surgery,
with total laryngectomy reserved for recurrences.
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