Vulvar neuroendocrine carcinoma: a case report and literature review

Background: Vulvar neuroendocrine neoplasms are extremely rare and reported only in case reports. Diagnosis and treatment are difficult because of the rarity of these tumors. This report describes a rare case of vulvar neuroendocrine carcinoma (NEC) and reviews the available literature on neuroendocrine neoplasms of the vulva to inform the clinical management of this rare tumor. Compared with similar cases that also with vulvar NEC, our patient received only vulvar wide local excision with no postoperative therapy and had good outcome until the next 17 months follow-up time. Case Description: A 47-year-old woman presented to a local hospital with a 1-month history of a vulvar mass that had progressively enlarged over the preceding 2 months. She underwent vulvar wide local excision at the hospital. The pathological results showed high-grade NEC. The patient then sought treatment at our hospital. We performed vulvar wide local excision. The second pathological examination showed no residual tumor. The patient did not receive any postoperative therapy and was alive with no recurrence 17 months after the surgery. We describe the clinical characteristics of the 29 cases identified in the literature using a Pubmed search. Conclusions: In summary, vulvar NEC is a rare disease. Our case and the reviewed cases further our understanding of the clinical presentation, diagnosis, and treatment of this rare disease. Due to the limited number of available studies with a sufficient follow-up period and large patient sample, more cases should be included in the future to help establish new treatment guidelines.