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

Crit Rev Oncol Hematol. 2024 Jan:193:104201. doi: 10.1016/j.critrevonc.2023.104201. Epub 2023 Nov 17.

Abstract

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.

Keywords: Gynecologic oncology; Gynecologic surgery; Neuroendocrine neoplasms; Neuroendocrine vulvar carcinoma; Vulvar tumors.

Publication types

  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Humans
  • Lymph Node Excision
  • Middle Aged
  • Neoplasm Staging
  • Neuroendocrine Tumors* / pathology
  • Vulva / pathology
  • Vulva / surgery
  • Vulvar Neoplasms* / diagnosis
  • Vulvar Neoplasms* / epidemiology
  • Vulvar Neoplasms* / therapy