A rare rarity: Neuroendocrine tumor of the esophagus

Elisa Giannetta, Valentina Guarnotta, Francesca Rota, Federica de Cicco, Federica Grillo, Annamaria Colao, Antongiulio Faggiano

Research output: Contribution to journalReview articlepeer-review


Esophageal Neuroendocrine tumors (NETs) are rare, aggressive and lacking specific symptoms. This causes a diagnostic delay, worsening the prognosis. Numerous cases are reported in literature, without a consensus on the management. Our aim was to clarify epidemiology, clinical presentation, diagnostic, therapeutic management of esophageal NETs. Extensive literature search identified a total of 226 articles. One hundred twenty-five articles (n = 1676) met the inclusion criteria, showing that: the incidence of esophageal NET varies geographically; men (60–70 years) are more affected; smoking and alcohol abuse are the major risk factors; dysphagia, weight loss, appetite loss are the most common clinical features. The histotypes include high-grade small and large cell esophageal carcinomas and low-grade carcinoid tumors. Mixed neuroendocrine/non-neuroendocrine neoplasms are the most common. Often the diagnosis occurs randomly on endoscopic examination. Circulating markers, functional combined with conventional imaging contributes to the diagnosis and management. Treatment depends on type, grade and stage of the tumor.

Original languageEnglish
Pages (from-to)92-107
Number of pages16
JournalCritical Reviews in Oncology/Hematology
Publication statusPublished - May 1 2019


  • Esophageal NEC
  • Gastroenteropancreatic NET
  • Large cell esophageal NEN
  • Small cell carcinoma

ASJC Scopus subject areas

  • Hematology
  • Oncology


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