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.