Primary neuroendocrine neoplasms of the vulva: A review of the MITO rare cancer group

Gynecological neuroendocrine neoplasms are rare entities and can be divided into two groups: carcinoids and neuroendocrine carcinomas. Due to their rarity their management is not standardized. The aim of this work is to summarize and discuss the current literature evidence on this pathology. A scoping literature review was performed in multiple databases. Thirty-one studies were included: 30 case reports and one case series. Patients’ age ranged between 28 and 92 years. Surgery was the most used treatment and the surgical approach included local excision (N = 16/31; 51.6%) with (N = 5/16; 31.25%) or without (N = 11/16; 68.75%) inguinal lymphadenectomy. Adjuvant radiotherapy was delivered in 12 (38.7%) cases; instead, platinum-based therapies were frequently used when chemotherapy was chosen for adjuvant treatment. The overall survival ranged between 20 days to 4 years. However, further research is needed; currently, multimodal approach including surgery, chemotherapy and radiotherapy appeared safe and feasible for the treatment of these rare and aggressive diseases. [Display omitted] •Primary neuroendocrine neoplasms of the female genital tract usually affect the cervix and ovary, occasionally the vulva.•Vulvar neuroendocrine neoplasms’ optimal management is not standardized and prognosis data are lacking.•An approach including surgery, medical and radiation treatments seem to be safe and feasible for these rare and aggressive diseases.