Neuroendocrine carcinoma of the endometrium: A very rare gynecologic malignancy

J Gynecol Obstet Hum Reprod. 2021 May;50(5):101897. doi: 10.1016/j.jogoh.2020.101897. Epub 2020 Aug 19.

Abstract

Objective: To investigate the clinicopathologic characteristics, prognostic factors, outcome, and treatment of the neuroendocrine carcinoma (NEC) of the endometrium.

Materials and methods: We retrospectively reviewed the clinicopathologic and survival data of 10 patients who underwent surgery for NEC. The patients were collected between 1999 and 2017 from four referral centers in Turkey.

Results: The median age of patients was 67 years (range: 34-75 years). The NEC of endometrium consist of 9 cases with small cell carcinoma (SC) NEC (two with mixed histotypes), and one with a large cell (LC) NEC. According to FIGO 2009 criteria, 70 % (7/10) of patients had advanced stage (III and IV) disease. All patients except one underwent surgical staging, eight patients received platinum-based chemotherapy (CTX) and of 6 those were additionally treated with radiotherapy (RT). Four patients died of disease ranging from 2 to 10 months and six were alive 12-72 months with no evidence of disease. In addition, 4 SC NEC cases raised in polypoid features had no evidence of disease from 24 to 72 months.

Discussion: NEC of the endometrium is a rare disease with poor prognosis, which frequently diagnosed in advanced stages. The main treatment modality was the administration of platinum-based CTX as an adjuvant to surgery or surgery and RT. Our result suggests that the polypoid feature of the tumor might be one of the best predictors for the prognosis of SC NEC.

Keywords: Clinicopathologic features; Endometrium; Neuroendocrine carcinoma.

MeSH terms

  • Adult
  • Aged
  • Carcinoma, Large Cell* / complications
  • Carcinoma, Large Cell* / mortality
  • Carcinoma, Large Cell* / pathology
  • Carcinoma, Large Cell* / therapy
  • Carcinoma, Neuroendocrine* / complications
  • Carcinoma, Neuroendocrine* / mortality
  • Carcinoma, Neuroendocrine* / pathology
  • Carcinoma, Neuroendocrine* / therapy
  • Carcinoma, Small Cell* / complications
  • Carcinoma, Small Cell* / mortality
  • Carcinoma, Small Cell* / pathology
  • Carcinoma, Small Cell* / therapy
  • Combined Modality Therapy / methods
  • Endometrial Neoplasms* / complications
  • Endometrial Neoplasms* / mortality
  • Endometrial Neoplasms* / pathology
  • Endometrial Neoplasms* / therapy
  • Female
  • Humans
  • Middle Aged
  • Prognosis
  • Rare Diseases*
  • Retrospective Studies
  • Treatment Outcome