discussion
SCCC is a relatively rare subtype of cervical malignancy. It can be
categorized as small cell carcinoma of extrapulmonary origin. It has
been increasingly recognized as a clinicopathological entity with
biological behavior and prognosis distinct from small-cell lung
carcinoma (SCLC)(10). Small-cell carcinomas resemble small-cell
carcinomas of the lung and are made up of small tumor cells
that have scanty cytoplasm, small round to oval nuclei, and high mitotic
activity; they frequently display neuroendocrine features(11).Almost all
sccc are immunoreactive for keratin and epithelial membrane antigen and
at least one marker of neuroendocrine differentiation is expressed in 88
to 100 percent of cases (including neuron-specific
enolase,synaptophysin,CGA and CD 56) (22). The clinical course of these
tumors is known to be aggressive in general, with early dissemination
and frequent recurrences. Although chemotherapy seems to be an effective
therapeutic modality as in SCLC, surgery and radiation therapy may also
play an important role depending on the stage or primary site (12).
A large case series of extrapulmonary small cell carcimas from England
identified 76 cervix cases out of 1618 (4,69%)(13).Other small series
have reported different numbers. For example a case series from South
Korea published in 2004 reported a 29% rate for a cervical site (14)
As previously stated, presentation and clinical manifestations of SCCC
is the same as other cervical cancers.
Multiple parameters have been noted as prognostic. In a review of 188
patients authors concluded that use of adjuvant chemotherapy or
chemoradiation was associated with higher survival in small cell
cervical cancer patients (15). In another review of 290 patients from
the Surveillance, Epidemiology, and End Results database On
multivariable analysis, age, stage, and race were prognostic for
survival in women with small cell carcinoma (16).Other series have
listed advanced disease(17,18),smoking(18),lymph node metastasis (19)and
hematogenous metastasis(20) as prognostic factors.
Regarding its etiologic factors a recent meta-analysis of 143 studies
revealed HPV-16 and HPV-18 to be cause of most small cell carcinomas of
cervix (21).
Most oncologists favor the use of combined modality therapy (surgery
followed by chemotherapy or combined chemoradiotherapy) for early-stage
potentially resectable disease, definitive chemoradiotherapy for
locoregionally advanced unresectable but nonmetastatic disease, and
palliative chemotherapy alone for those with metastatic disease, using
chemotherapy regimens that are typically used for small cell lung cancer
(23). In a recent systematic review of literature Tempfer et al. pointed
to similar trends in practice and concluded that cisplatin/carboplatin
with etoposide alone or in combination with other agents is the most
common regimen (24). Since SCCC is a rare disease, most series have
small numbers and no prospective trial has been done to this date, data
are limited to guide decision-making and there is no consensus as to
optimal management (22). Treatment generally considers the treatment
options for cervical cancer, particularly chemotherapy, which have been
largely extrapolated from the experience with small cell lung cancer
(7).
In a review of 100 extrapulmonary small cell carcinoma authors concluded
that definitive chemoradiation was associated with improved outcomes.
Additionally prophylactic cranial irradiation improved overall survival
but the benefit was less than SCLC (25).
In conclusion, small cell carcinoma of cervix is an aggressive form of
cervical cancer with poor prognosis. Prognosis is poor and optimal
treatment remains undefined.