Small Cell Carcinoma of the Vagina: First Systematic Review of Case Reports and Proposal of a Management Algorithm

Abstract

ObjectivesSmall cell carcinoma of the vagina (SmCCV) is an extremely rare disease. Evidence-based data and specific guidelines are lacking. We conducted the first systematic review of case reports to provide the most overall picture of SmCCV.Materials and MethodsLiterature search in PubMed and Scopus was performed using the terms “small cell carcinoma” and “vagina.” English-language case reports of primary SmCCV up to January 2022 were included.ResultsTwenty-nine articles describing 44 cases met our inclusion criteria. We report a new case of our hospital. The global median overall survival (mOS) was 12.00 months (95% CI = 9.31–14.69). The mOS was not reached for stage I, and it was 12.00, 12.00, 9.00, and 8.00 months for stages II, III, IVA, and IVB, respectively (statistically significant differences between stage I and stages II, III, or IVA [log rank p = .003–.017]). Thirty-five cases received local treatments (77.8%). The mOS of patients treated with surgery ± complementary chemotherapy, radiotherapy ± complementary chemotherapy, chemoradiation ± complementary chemotherapy, and surgery + radiotherapy ± complementary chemotherapy were 11.00, 12.00, 17.00, and 29.00 months, respectively. The use of adjuvant or neoadjuvant chemotherapy (64.5%, mostly platinum + etoposide) showed longer mOS (77.00 vs 15.00 months). Four of 5 tested cases presented human papillomavirus infection, 3 of them presenting type 18.ConclusionsSmall cell carcinoma of the vagina shows dismal prognosis. Multimodal local management plus complementary chemotherapy seems to achieve better outcomes. Human papillomavirus could be related to the development of SmCCV. A diagnostic-therapeutic algorithm is proposed.

Small Cell Neuroendocrine Carcinoma of the Vagina: A Rare Presentation

“…Neuroendocrine tumors (NET) occur in a spectrum of malignancies; among them, small-cell cancer is the most aggressive. Often, small cell carcinomas (SmCCs) arise in the lung and only 5% are extrapulmonary [ 2 ]. Common in postmenopausal females, the age group for vaginal SmCCs is 38-74 years.…”

Small‐cell neuroendocrine carcinoma of the female genital tract: A comprehensive overview

“…Immunohistochemistry shows positivity for one or more neuroendocrine markers, including chromogranin A and synaptophysin. 1,106,111 Additionally, there are reports of positive staining for CK20, CK7, CD57, and AE1/AE3 [112][113][114] and negative reaction for CD56 and p63. 112 Interestingly, TTF1 can be positive in primary SCNECVa, so it is not a reliable marker to differentiate primary from metastatic SCNECVa.…”

“…In a recent systematic review, multimodality treatment with surgery and radiotherapy with or without chemotherapy was associated with the longest median overall survival of 29 months. 112 The type of surgery depends on the locoregional extent of the tumor, ranging from local resection in small localized lesions to pelvic exenteration in advanced cases. The role of lymphadenectomy is not clear and is usually performed in more advanced stage tumors.…”

“…120 However, based on the recent systematic review by Capote et al, four of the five tested cases presented HPV, with three of them presenting type 18. 112 It is not shown that involvement of a particular vaginal segment or amount of vaginal surface area involved with the tumor affects patients' outcome. 100 Chen et al on a series of 16 cases, all tumor cells in this subcategory were found to contain the viral genome.…”

“…Various treatment modalities, including surgery, chemotherapy, and radiotherapy, either alone or in combination, have been used. In a recent systematic review, multimodality treatment with surgery and radiotherapy with or without chemotherapy was associated with the longest median overall survival of 29 months 112 . The type of surgery depends on the locoregional extent of the tumor, ranging from local resection in small localized lesions to pelvic exenteration in advanced cases.…”