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Esophageal neuroendocrine tumor

MedGen UID:
459486
Concept ID:
C2987260
Neoplastic Process
Synonyms: Esophageal NEN; esophageal NEN; Esophageal NET; esophageal NET; Esophageal neuroendocrine neoplasm; esophageal neuroendocrine neoplasm; Esophageal Neuroendocrine Tumor; esophageal neuroendocrine tumor; Esophageal Well Differentiated Endocrine Tumor; esophageal well differentiated endocrine tumor; Esophageal Well Differentiated Endocrine Tumor/Carcinoma; esophageal well differentiated endocrine tumor/carcinoma; esophageal well differentiated endocrine tumour; esophagus NET; esophagus neuroendocrine neoplasm; esophagus neuroendocrine tumor; esophagus neuroendocrine tumor, well differentiated, low or intermediate grade; NEN of esophagus; NEN of oesophagus; neuroendocrine neoplasm of esophagus; Neuroendocrine neoplasm of esophagus; neuroendocrine neoplasm of oesophagus; neuroendocrine tumor of esophagus; neuroendocrine tumour of oesophagus; oesophagus NET; oesophagus neuroendocrine neoplasm; oesophagus neuroendocrine tumor, well differentiated, low or intermediate grade; oesophagus neuroendocrine tumour
 
Monarch Initiative: MONDO:0003649
Orphanet: ORPHA506136

Definition

A group of esophageal epithelial neoplasms characterized by neuroendocrine differentiation, comprising well-differentiated neuroendocrine tumors (NETs), poorly differentiated neuroendocrine carcinomas (NECs), and mixed neuroendocrine-non-neuroendocrine neoplasms, an umbrella category including mixed adenoneuroendocrine carcinoma. The tumors typically occur in the lower esophagus, often in association with Barrett mucosa. NECs may also arise in other parts of the esophagus. On endoscopy, NETs usually appear as small polypoid or nodular submucosal masses, while NECs are large, infiltrative, and ulcerated. Patients most commonly present with dysphagia, pain, weight loss, and sometimes melena. Metastatic NETs may be associated with carcinoid syndrome. [from ORDO]

Professional guidelines

PubMed

Ma Z, Cai H, Cui Y
Tumour Biol 2017 Jun;39(6):1010428317711313. doi: 10.1177/1010428317711313. PMID: 28653897

Recent clinical studies

Etiology

Wu IC, Chu YY, Wang YK, Tsai CL, Lin JC, Kuo CH, Shih HY, Chung CS, Hu ML, Sun WC, Wang JP, Wang HP
J Formos Med Assoc 2021 Jan;120(1 Pt 2):508-514. Epub 2020 Jun 27 doi: 10.1016/j.jfma.2020.06.024. PMID: 32600867

Diagnosis

Aoki H, Omori Y, Katanuma A
Clin Gastroenterol Hepatol 2022 Apr;20(4):e651-e652. Epub 2021 Mar 1 doi: 10.1016/j.cgh.2021.02.039. PMID: 33662595
Filizoglu N, Ozguven S
Clin Nucl Med 2021 Jul 1;46(7):e393-e394. doi: 10.1097/RLU.0000000000003551. PMID: 33630804
Wu IC, Chu YY, Wang YK, Tsai CL, Lin JC, Kuo CH, Shih HY, Chung CS, Hu ML, Sun WC, Wang JP, Wang HP
J Formos Med Assoc 2021 Jan;120(1 Pt 2):508-514. Epub 2020 Jun 27 doi: 10.1016/j.jfma.2020.06.024. PMID: 32600867
Chin YP, Lai WF, Chiang MT, Chang SC
Medicine (Baltimore) 2017 Dec;96(50):e9282. doi: 10.1097/MD.0000000000009282. PMID: 29390385Free PMC Article
Ma Z, Cai H, Cui Y
Tumour Biol 2017 Jun;39(6):1010428317711313. doi: 10.1177/1010428317711313. PMID: 28653897

Prognosis

Filizoglu N, Ozguven S
Clin Nucl Med 2021 Jul 1;46(7):e393-e394. doi: 10.1097/RLU.0000000000003551. PMID: 33630804
Wu IC, Chu YY, Wang YK, Tsai CL, Lin JC, Kuo CH, Shih HY, Chung CS, Hu ML, Sun WC, Wang JP, Wang HP
J Formos Med Assoc 2021 Jan;120(1 Pt 2):508-514. Epub 2020 Jun 27 doi: 10.1016/j.jfma.2020.06.024. PMID: 32600867
Chin YP, Lai WF, Chiang MT, Chang SC
Medicine (Baltimore) 2017 Dec;96(50):e9282. doi: 10.1097/MD.0000000000009282. PMID: 29390385Free PMC Article
Ma Z, Cai H, Cui Y
Tumour Biol 2017 Jun;39(6):1010428317711313. doi: 10.1177/1010428317711313. PMID: 28653897
Matsuda K, Sakurada K, Kokubo Y, Sato S, Nakazato Y, Kayama T
Brain Tumor Pathol 2014 Jul;31(3):192-7. Epub 2014 Feb 16 doi: 10.1007/s10014-014-0178-0. PMID: 24531830

Clinical prediction guides

Wu IC, Chu YY, Wang YK, Tsai CL, Lin JC, Kuo CH, Shih HY, Chung CS, Hu ML, Sun WC, Wang JP, Wang HP
J Formos Med Assoc 2021 Jan;120(1 Pt 2):508-514. Epub 2020 Jun 27 doi: 10.1016/j.jfma.2020.06.024. PMID: 32600867

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