International Journal of Otolaryngology and Head & Neck Surgery, 2013, 2, 259-262
Published Online November 2013 (http://www.scirp.org/journal/ijohns)
http://dx.doi.org/10.4236/ijohns.2013.26054
Open Access IJOHNS
Carcinosarcoma of the Hypopharynx—A Rare Entity with
Unique Surgical Procedure
Sangeet Kumar Agarwal*, Satinder Singh, Shalabh Sharma, Asish Kumar Lahiri
Department of ENT and Head, Neck Surgery, Sir Ganga Ram Hospital, New Delhi, India
Received August 12, 2013; revised September 10, 2013; accepted October 2, 2013
Copyright © 2013 Sangeet Kumar Agarwal et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Background: Carcinosarcoma of hypopharynx is a rare neoplasm with both malignant epithelial and mesenchymal
components. They are highly aggressive in nature with controversial histogenesis. To our knowledge, there is no case
reported of carcin osarcoma of hypopharynx managed w ith wide LASER excision and modified radical neck dissection
followed by chemorad iation. Method and Result: 42-year-old male presented complaints of foreign body sensation in
throat, and difficulty in swallo wing and hoarseness for 4 months. On examination, a large smooth globular mass filling
the supraglottis obscuring the view of endolarynx was noted and right side level-II cervical lymphadenopathy was
found. CECT neck revealed a large hypopharyngeal mass abutting the supraglottis. Microlaryngoscopic biopsy was
reported as high grade carcinosarcoma and PET scan was done after 1 week which revealed no any distant metastasis.
The patient was taken for transoral pulsed carbon dioxide LASER excision with modified radical neck dissection, and
postoperative chemoradiation was done and after two years of closed follow-up MRI was done which revealed no dis-
ease and the patient is absolu tely free of disease till now. Conclusion: It was a rare case which was managed with com-
plete LASER excision with neck dissection and postoperative chemoradiation and the patient is absolutely free of dis-
ease till now after 2 years of follow-up.
Keywords: Carcinosarcoma; PET Scan; LASER
1. Introduction
Carcinosarcoma is a rare neoplasm with both malignant
epithelial and malignant mesenchymal components. Car-
cinosarcoma 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 fre-
quently the oral and nasal cavities and the esophagus.
True carcinosarcomas of the hypopharynx and larynx
represent less than 1% of all malign ant neoplasms in this
area. Carcinosarcomas are aggressive regardless of their
origin, but differentiating their origin is important be-
cause the prognosis also depends on the site of origin [1].
The histogenesis is controversial and the most ac-
cepted theory is the differentiation of primitive blastic
mesenchymal cells that can mature and produce malig-
nant neoplasm of multiple differentiations [2].
To our knowledge, there is no case reported on carci-
nosarcoma of hypopharynx managed with wide LASER
excision and modified radical neck dissection followed
by chemoradiation.
2. Case Report
A 42 years old male presented with complain ts of foreign
body sensation in throat and hoarseness for last 4 months.
He also had difficulty in deglutition, which was more to
solids than to liquids for last 2 months. He was a chronic
smoker, betel nut chewer and had a history of alcohol
consumption regularly. He did not have difficulty in
breathing at presentation. Rigid Hopkins Rod Laryngo-
scopy showed a large smooth globular mass filling the
supra-glottis, endolarynx could not be seen and origin of
the mass could not be made out. A single right side level
II Cervical lymphnode was palpable which was ap-
proximately 1.5 cm × 1.5 cm in size and firm in consis-
tency was noted. Contrast Enhanced Computed Tomo-
grahy scan of neck was done which revealed enhancing
mass inseparable from the right aryepiglottic fold and the
right posterolateral pharyngeal wall abutting the epiglot-
tis with enlarged necrotic right upper deep cervical lymp h
*Corresponding author.

S. K. AGARWAL ET AL. 260
nodes (Figure 1).
In view of the compromised airway, temporary tra-
cheostomy followed by microlaryngoscopic excision
biopsy was done using a Weerda distending suspension
laryngoscope, the mass was found to be attached with a
broad based pedicle to right pharyngoepiglottic fold go-
ing down upto superior border of lateral wall of pyriform
sinus. The pedicle base was cauterized with a bipolar
forceps prior to excision. Biopsy (S1607/11) was re-
ported as high grade carcinosarcoma.
Gross pathological examination showed a single bulky
nodular piece of tissue measuring 3 × 2.5 × 2 cm with
smooth outer surface, showed focal areas of necrosis. Cut
surface was grey white, lobulated and gelatinous.
Microscopically nodular tumor with largely ulcerated
overlying epithelium was seen. The tumor shows bi-
phasic pattern having both epithelial as well as stromal
components. Epithelial component showed poorly dif-
ferentiated squamous cell carcinoma in most places while
in occasional foci, it showed squamous differentiation
with keratin pearls. Spindle cell component showed
prominent osteoid deposition in lace like pattern (depos-
ited by tumor cells). Calcification was also prominent
within the tumor along with focally scattered inflamma-
tory cells. Few scattered giant cells are also seen. Mito sis
was very high (25 - 30/10 HPF) with many atypical mi-
tosis. Necrosis is confined in the periphery of the tumor.
Immunohistochemistry examination revealed that the
epithelial component of the tumor cells were strongly
positive of EMA and bcl2. Occasional cells stain Cy-
tokeratin. Spindle cells are positive for vimentin. CD34
and S-100 stains are negative. SMA shows nuclear posi-
tivity in most of the cells (both epithelial and stromal)
(Figure 2).
PET scan was done after 1 week to rule out distant
metastasis (Figure 3).
Patient was taken up for transoral laser excision of the
base of the previously excised pedicle. The slough cov-
ered areas left behind after the excision biopsy were
marked out using LASER with margin of 5 mm. The
edge of the area was held with forceps and pulled medi-
ally and dissected away from the muscular layer with the
pulsed CO2 LASER. This included the pharyngo-epi-
glottic fold and the right edge of the epiglottis. Right
modified radical neck dissection type I was done. Mar-
gins of the surgical resection were negative, out of total
44 neck nodes, 3 lymph nodes were invaded by tumor.
Patient was referred for chemotherapy and radiotherapy.
Image guided intensity modulated radiation therapy
(IGRT), total dose of 66 Gy was given in 33 fractions.
He also received concurrent chemotherapy with inj.cis-
platinum 70 mg once a week for 6 weeks. He tolerated
the CT and RT well. The latest Magnetic resonance im-
aging of neck done after 1.5 years reveals no evidence of
Figure 1. Coronal cut of CECT scan showing globular le-
sion arising from lateral pharyngeal wall obscuring the
lumen.
Figure 2. Histopathological slide initially stained with he-
matoxylin and eosin (H & E) and finally stained with
vimentin and S-100, showing epithelial and mesenchymal
components.
disease remnant and patient is absolutely free of disease
till now (Figure 4).
3. Discussion
Carcinosarcoma is an unusual and rare neoplasm of the
upper aerodigestive tract. The diagnosis implies a bi-
phasic neoplasm containing both mesenchymal and
epithelial elements, each of wh ich displays the histologic
and biologic criteria of malignancy [2].
In 1865, Virchow named the rare malignant neoplasm
of squmaous and sarcomatous cell types “carcinosar-
coma”, since then it has also been called pseudosarcoma,
spindle cell carcinoma and sarcomatoid carcinoma. This
mixed type of tumour with a combination of carcinoma-
tous and sarcomatous cell types has been found in can-
cers of the uterus, vagina, lungs, oral cavity, larynx,
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S. K. AGARWAL ET AL. 261
Figure 3. Coronal cut of PET-CT scan showing increased
uptake of 18-FDG (fluro deoxy glucose) in left upper jugu-
lodigastric lymphnode.
Figure 4. Coronal cut of contrast enhanced MRI showing
post radiotherapy edema of the pharynx with partial ab-
sence of epiglottis without showing recurrence.
thyroid, urinary tract and esophagus [3,4].
This tumor is uncommon in the hypopharynx and is
commoner elsewhere in the upper aerodigestive tract,
and in decreasing order of frequency—larynx, oral cavity,
hypopharynx & pyriform fossa, sinonasal tract and oro-
pharynx. It has an association with smoking (87%), al-
cohol (48%) and history of radiation therapy. The tumor
may recur locally (18%) or have distant metastasis (14%)
[5]. The lesion is a polypoidal, exophytic, or fungating
mass. Surface ulceration is common with these tumors
[6].
The exact histogenesis has been a source of debate.
Four pathogenic explanations have been proposed. First,
the tumor may be a carcinosarcoma, which is a collision
tumor. Second, the tumor may be a squamous cell carci-
noma with an atypical reactive stoma (pseudosarcoma).
Third, a malignancy arises in an embryonic rest of
epithelial and mesenchymal tissues. And fourth, the tu-
mor de-differentiating results in spindle cell morphologic
features (sarcomatoid carcinoma) [2].
In case of carcinosarcoma, there is primitive mesen-
chymal blastic cell differentiation that can mature and
produce malignant neoplasms of multiple differen tiations.
True sarcoma is the major component with a high mitotic
index, cell pleomorphism, and dysplasia to invasive car-
cinoma or adeno carcinoma, and no areas of transition are
observed.
On immunohistochemistry, epithelial component is
positive to cytokeratin, and mesenchymal component is
positive to vimentin and other markers like S-100, actin,
desmin etc. depending on tumour’s origin.
In the present case, the epithelial component of the
tumor cells is strongly positive to EMA and bcl2. Occa-
sional cells stain cytokeratin. Spindle cells are positive to
vimentin. CD34 and S-100 stains are negative. SMA
shows nuclear positivity in mo st of the cells (both epith e-
lial and stromal).
Diagnosis is usually established early on the basis of
the clinical presentation which is generally characterized
by the presence of an exophytic tumour that quickly
causes obstruction as a result of its rapid growth, espe-
cially the growth of the mesenchymal component. A pa-
tient with suspected carcinosarcoma should undergo a
deep biopsy of a non-ulcerated area because when ul-
ceration is present, the affected area can become over
infected which can lead to a f a lse negative biopsy [1] .
The differential diagnosis of spindle cell carcinoma
includes rare sarcoma such as fibrosarcoma, malignant
fibrous histiocytoma, and another biphasic tumor-syno-
vial cell sarcoma. Also hypocellular reactive processes
such as radiation changes or granulation tissue must be
distinguished from spindle cell carcinoma. Immunostains
for keratin are often useful in diagnosis. The presence of
keratin positivity and nondiploid DNA content in the
spindle cell population supports the neoplastic epithelial
origin of these tumors (sarcomatoid carcinoma). The
overall tumor behavior and surgical therapy appeared to
be comparable with those of squamous cell carcinoma at
a similar stage [2].
There is no consensus as to the most appropriate ther-
apy for carcinosarcoma. The most common treatment is
total laryngectomy complemented in some cases by ad-
juvant chemotherapy and/or radiotherapy because the
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S. K. AGARWAL ET AL.
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262
prognosis is dependent on the type of malignant mesen-
chymal component. [7] In accordance with the previous
literature, surgical therapy is the primary mode of treat-
ment of these lesions. Conservational operations are per-
formed when indicated. Various methods include con-
servation partial laryngectomies like vertical and hori-
zontal partial laryngectomies and in some cases more
radical surgeries like supracricoid partial laryngectomy
with cricohyoidpexy and total laryngectomies with or
without neck dissections are done primarily for hypo-
pharyngeal, supraglottic, advanced glottic and transglot-
tic tumors. Another mode of treatment is chemotherapy
and radiotherapy alone or combined with surgery. Con-
servational surgeries and non-surgical therapy are func-
tionally preservative methods but prognostically less
favourable methods. As the metastasis has been reported
for such type of carcinomas, it seems to be reasonable to
use combined therapy, as neck dissections are associated
with better cure rates, and elective neck dissection must
be performed at the time of the initial treatment of a non-
epithelial neoplasm, therefore the most common treat-
ment is definitive surgery of that organ complemented
with neck dissection in some cases by adjuvant chemo-
therapy and/ or radiotherapy [1,7- 9].
Prognosis of true carcinosarcoma of the hypopharynx
is very poor because it is very aggressive and highly me-
tastatic in nature, and most of the cases died due to dis-
tant metastasis to lungs. The exact prognosis of these
tumours is not known.
The most important prognostic factors appear to be
location {hypopharynx versus larynx and supraglottic
versus glottic}, the size of the tumor and the presence of
cervical metastasis.
In the present case, the tumour was excised from the
hypopharynx with the help of pulsed CO2 LASER with
modified radical neck dissection type 1, and postopera-
tive co ncu rrent radi othe rapy a nd c hemot hera py we re gi ven.
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