Advanced neuroendocrine carcinoma (Merkel cell carcinoma) of the vulva: a case report and literature review

Abstract

Neuroendocrine carcinomas (Merkel cell carcinomas) of the vulva are extremely rare tumours, with very few cases reported to date. Herein, a primary neuroendocrine carcinoma (Merkel cell carcinoma) of the vulva is reported. A 34-year-old HIV-positive female on antiretroviral therapy presented with a four-month history of a right-sided vulval mass. She underwent surgical excision of a histologically confirmed neuroendocrine carcinoma. Twenty-four weeks after surgery, she died. This case illustrates the importance of a broad differential diagnosis for neoplasms in the usual sites, and the aggressive nature of this tumour, which to date has had limited effective treatment options.