Capote S, Domènech M, Valdivieso L, Tuset V, Sanchez M, Carballas E, Teruel I, Durany D, Moragas G, Molina C, Lleberia J, Martínez-Roman S, and Romeo M
Objectives: Small 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 Methods: Literature 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., Results: Twenty-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., Conclusions: Small 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., Competing Interests: M.D. has received honoraria from advisory board (Novartis) and travel grants for attending to medical congresses (Seagen, Roche, Bristol Meyers Squibb, Novartis, MDS, AstraZeneca, Lilly). I.T. has received honoraria for speaking (Novartis) and inscription grant for attending medical congresses (AZ) in the last year. S.M.R. has received honoraria from advisory boards AstraZeneca, TESARO, and ROCHE. M.R. has received honoraria from advisory boards (AstraZeneca/MSD, GSK, Merck and Clovis) and travel grants for attending medical congresses (Pfizer) in the last year. The other authors have declared they have no conflicts of interest., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.)