2016, Volume 32, Number 2, Page(s) 122-125
Mucoepidermoid Carcinoma in Carcinosarcoma of the Lung - A Rare Combination
Meetu AGRAWAL, Tara Roshni PAUL, Shantveer G. UPPIN, Sundaram CHALLA
Department of Pathology, Nizam's Institute of Medical Sciences, HYDERABAD, INDIA
Keywords: Lung, Carcinosarcoma, Mucoepidermoid carcinoma
Carcinosarcomas of the lung are uncommon neoplasms and are
presently categorized along with sarcomatoid carcinomas in the
WHO 2004 classification. Morphological recognition of individual
components in a surgical specimen is usually straightforward. The
respiratory epithelium is also a rare site for salivary gland type
tumors. A 72-year-old lady who was investigated for an episode of
hemoptysis was found to have a well circumscribed intraluminal
tumor. The histo-morphological features were unique and composed
of mucoepidermoid carcinoma and undifferentiated sarcoma. To the
best of our knowledge, this unusual morphological combination has
never been reported in literature.
Carcinosarcoma is defined as a tumor composed of epithelial
and mesenchymal elements, both components being
malignant (WHO 2004)1,2
. Though carcinosarcoma
has been described at all sites where a carcinoma can occur
(uterus, breast, thyroid, esophagus, lung), it is overall a rare
diagnosis. In the lung, it accounts for less than 0.3% of all
lung cancers and has been grouped along with sarcomatoid
. Primary salivary gland type lung tumors are
a distinct rare category of lung malignancy with well-defined
. Mucoepidermoid carcinoma forms the
second commonest type (after adenoid cystic carcinoma)
in this category. However, it has never been reported as a
component of carcinosarcoma, where conventional nonsmall
cell lung cancers are commoner. This case is reported
here because of an uncommon morphological diagnosis.
A 72-year-old lady presented with a single episode of
hemoptysis 15 days back. There had been no prior episodes,
fever, breathlessness, cough, expectoration, chest pain or loss of weight. General examination was unremarkable.
Respiratory rate was 14/minute. Ultrasound abdomen
showed all viscera in their normal anatomical condition.
Chest radiograph and CT scan revealed a single intrabronchial
mixed density mass in the right upper bronchus
with no evidence of atelectasis. FNA done outside this
hospital was suggestive of a “malignant spindle cell tumor”.
Evaluation for any primary tumor elsewhere and workup
for metastasis was negative. She underwent right upper and
middle lobectomy along with hilar lymph node clearance
for this lung tumor.
Surgical specimen: The right upper main bronchus
was dilated with an intraluminal growth measuring
4.8x3x1.2cm. Cut surface was variegated with hemorrhagic
areas and patchy consolidation in the distal lung (Figure
1A). Mapped sections from the specimen submitted
showed the lesion composed of two discreet areas with
different morphologies. There was an epithelial component
composed of a variable admixture of nests and solid islands
of squamous, intermediate and mucin producing cells
(Figure 1B-D). The acidic mucin was stained positively by Alcian PAS stain. The mesenchymal component was
composed of short and long interlacing fascicles and
focal sheet like arrangement of spindle shaped cells with
irregularly interspersed giant cells. The cells had moderately
pleomorphic vesicular nuclei, conspicuous nucleoli and
scant to moderate cytoplasm (Figure 1E). Interspersed
tumor giant cells were seen. Mitosis was 4-5/10HPF. Areas
of hemorrhage were seen but no osseous or cartilaginous
component was noted. Repeated deeper sections and
extensive grossing till exhaustion of the tumor did not yield
any differentiation of the sarcomatous component.
Click Here to Zoom
|Figure 1: a) Gross specimen shows intra-bronchial mass with heterogeneous areas. H&E stained photomicrographs show
b) squamous and intermediate cells (H&E; x200), c) mucin producing cells with Alcian blue positivity (inset), d) intermediate cells and
e) sarcomatous areas with numerous giant cells, inset shows mitotic activity.
The remaining lung parenchyma showed focal organized
fibrinous exudates with mononuclear inflammatory cells. Hilar lymph nodes were negative for metastasis.
Immunohistochemistry was done with a panel comprising
of vimentin, pancytokeratin (CK), Ki67 and thyroid
transcription factor (TTF-1). CK was positive in epithelial
and vimentin was strongly expressed in the mesenchymal
component. Ki67 in the mesenchymal component was
28%, thus confirming its sarcomatous nature (Figure 2
A-C). TTF-1 was positive in pulmonary epithelial cells but
negative in the neoplastic component. Desmin, myogenin,
S-100, smooth muscle actin, CD34 were negative.
Click Here to Zoom
|Figure 2: Immunohistochemistry with a) Pancytokeratin showing positivity in epithelial component. b) Vimentin is positive in
sarcomatous component, inset shows negative immunostaining in epithelial component and c) Ki67 index of 28% indicating high
proliferative capacity in neoplastic spindle cells.
A diagnosis of pulmonary carcinosarcoma with undifferentiated
sarcoma was given. No adjuvant therapy was given
as the tumor was entirely intra-bronchial and there was no evidence of metastasis elsewhere. The patient is asymptomatic
after 7 months of follow-up.
Carcinosarcomas are rare tumors accounting for less
than 0.3% of all lung malignancies4
. The commonest
carcinomatous component is a squamous cell carcinoma
and sarcomatous component is usually an osteosarcoma,
chondrosarcoma or a rhabdomyosarcoma6
. Also, salivary
gland tumors in the lung have a histologic resemblance to
their glandular counterparts and are well described but rare5
. This peculiar case is probably the first of its kind where a
mucoepidermoid carcinoma was part of a carcinosarcoma.
Despite extensive sampling, we could not characterize the
sarcoma into any morphological category and it remained
an undifferentiated sarcoma.
Salivary gland type tumors in the lung form a distinct
subset and are thought to arise from the submucosal
glands of the tracheobronchial tree. The largest reported
series included 62 cases. The commonest type was adenoid
cystic carcinoma followed by mucoepidermoid carcinoma. Prognostically, the patients fare well when compared to
conventional non-small cell lung cancers7.
Most carcinosarcomas are seen in males (male-to-female
ratio= 7:1) in the sixth decade of life4,6. Associations
have been reported with smoking and asbestosis8. In
a large series of pulmonary carcinosarcomas by Davis
et al, 62% were endobronchial and 38% were peripheral
in location4. Interestingly, location is important as the
centrally located endobronchial tumours tend to present
early with cough, dyspnea or hemoptysis when compared
to their peripheral counterparts. In contrast, peripheral
tumors usually attain large size and present with metastasis
or invade the surrounding lung. Our patient was a nonsmoker
female and the tumor was endobronchial. It had
expanded the lumen of the bronchus but still presented early
with hemoptysis without any invasion of the pulmonary
parenchyma. These findings are in accordance with those
reported in literature.
The origin of carcinosarcomas is controversial. Theories
of its histogenesis have been proposed by Cohen-Salmon
et al. and include malignant change in a hamartoma, simultaneous epithelial and stromal malignancy, malignant
change in the stroma induced by carcinoma, connective
tissue metaplasia of epithelial cells and carcinomatous
change in a sarcoma9.
Squamous cell carcinoma (46%) followed by
adenocarcinoma (31%) and adenosquamous carcinoma
(19%) account for epithelial component in most pulmonary
carcinosarcomas. The sarcomatous component is usually
rhabdomyosarcoma, chondrosarcoma, osteosarcoma or a
combination of these10. In most instances, the sarcoma
is a poorly differentiated spindle cell sarcoma, which after
extensive sampling yields differentiation. For the present
case, we have undertaken extensive grossing, to the extent
of exhausting the entire tumor. Multiple serial and deeper
sections at various levels were studied. These did not reveal
any rhabdoid, osseous or cartilaginous component. To the
best of our knowledge this is the first case of carcinosarcoma,
with a combination of mucoepidermoid carcinoma and
pleomorphic undifferentiated sarcoma. Whether these
patients are amenable to surgical resection or not is of
little clinical significance. This is because in most of these
cases the diagnosis is incomplete before surgery. Though
uncommon, the tumors may metastasize; the epithelial
component metastasizing to regional lymph nodes and
the sarcomatous component to distant sites. Prognosis
is generally poor and general rules that are applied to
treatment of sarcomas must be followed11. Survival in
these patients is variable with a mean 5 year survival rate
being 21.3%7. The median survival in a series of 17
patients was 1 year4. For all tumors less than 3 cm in size,
and no metastasis, central location has a favorable outcome
when compared to peripheral tumors1,4. At greater than
6 cm size, patients have reduced survival7.
This case is reported here because of the unusual
morphological combination. To the best of our literature
search, it has never been earlier reported.
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