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Crit Rev Oncol Hematol. 2019 May;137:92-107. doi: 10.1016/j.critrevonc.2019.02.012. Epub 2019 Mar 2.

A rare rarity: Neuroendocrine tumor of the esophagus.

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Dept. of Experimental Medicine, "Sapienza" University of Rome, Italy. Electronic address:
Biomedical Department of Internal and Specialist Medicine (DIBIMIS), Section of Endocrine-Metabolic Diseases, University of Palermo, Italy.
Unit of Endocrinology, San Camillo-Forlanini Hospital, Rome, Italy.
Department of Clinical Medicine and Surgery, University "Federico II", Naples, Italy.
Pathology Unit, Department of Surgical Sciences and Integrated Diagnostics (DISC), University of Genova and Ospedale Policlinico San Martino, Genova, Italy.
Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo Studio e la Cura dei Tumori "Fondazione G. Pascale", IRCCS, Naples, Italy.


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.


Esophageal NEC; Gastroenteropancreatic NET; Large cell esophageal NEN; MANEC; Small cell carcinoma

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