Primary Small-Cell Neuroendocrine Carcinoma of the Vagina: A Case Report and Literature Review

Small-cell neuroendocrine carcinoma (SCNC) of the vagina is an exceedingly rare and highly aggressive tumor of the female genital tract. The vast majority of SCNCs occur outside the female genital tract, with gynecological involvement being remarkably rare. Despite modern advances in tumor genomic profiling, SCNC continues to demonstrate an aggressive clinical course and remains associated with an unfavorable prognosis. A 63-year-old woman presented to the Gynecology clinic with postmenopausal bleeding following a total abdominal hysterectomy with right salpingo-oophorectomy for uterine fibroids and a benign right ovarian cyst several years before. On assessment, a vaginal wall mass was discovered, and subsequent biopsy with histopathological assessment demonstrated an SCNC. Positron-emission tomography computed tomography confirmed primary vaginal SCNC with no metastatic foci. The patient subsequently underwent localized excision and was recommended for systemic treatment after a multidisciplinary team discussion. Following the first reported case many years ago, only a small number of cases have been documented in the medical literature. Due to the paucity of publications on SCNC of the vagina, there is currently no consensus on standard treatment protocols for this tumor. Therefore, we aim to raise awareness and research on this unique presentation of SCNC of the vagina.