胃腸神經內分泌瘤

Gastrointestinal Neuroendocrine Tumors

孔麒豪(Chi-Hao Kung) ； 歐建麟(Jian-Lin Ou) ； 陳保中(Bao-Chung Chen) ； 林榮鈞(Jung-Chun Lin)

《內科學誌》 29卷4期 (2018/08) Pp. 217-229

https://doi.org/10.6314/JIMT.201808_29(4).03

摘要

神經內分泌瘤（neuroendocrine tumor）簡稱為NET，為具有神經內分泌分化的表皮細胞腫瘤，最常發生在消化系統中。消化系統相關神經內分泌瘤分為胃腸神經內分泌瘤gastrointestinal NET（GI-NET），及胰臟NET。GI-NET以往稱為類癌（carcinoid），主要的症狀為類癌症候群相關的症狀。症狀的特異性低，常被誤認是其他病症。現有診斷工具為測量24小時尿中的5-氫氧靛基醋酸（5-Hydroxy indoleacetic acid，5-HIAA）、血清嗜鉻粒蛋白A（Chromogranin A，CgA）、影像、內視鏡檢查及放射性標記體抑素類似物成像。另外世界衛生組織（World Health Organization，WHO）在2017年做了病理診斷上的更新，以細胞分化程度、有絲分裂數及Ki67指數來做分類。治療則根據腫瘤大小、細胞分化程度、活動度及是否肝轉移來決定治療方式。本文回顧GI-NET之流行病學、臨床表現、診斷、分期及最新治療，使臨床醫師對GI-NET能有更多了解，以期能提高早期診斷率並使病人接受適當的處置及提高整體存活率。

關鍵字

神經內分泌瘤(Neuroendocrine tumor) ； 類癌(Carcinoid tumor) ； 類癌症候群(Carcinoid syndrome

並列摘要

Neuroendocrine tumors (NETs) are a genetically diverse group of malignancies that have neuroendocrine differentiation and occurs most often in the digestive system. Digestive system-associated NETs are classified into gastrointestinal (GI)-NETs and pancreatic NETs. GI-NET was formerly known as carcinoid which causes carcinoid syndrome as the main presentations. Because the symptoms are not specific, it is often misdiagnosed as other diseases. The current diagnostic tools include 24-hour urinary excretion of 5-hydroxyindoleacetic acid, serum chromogranin A, image, endoscopy, and somatostatin receptor scintigraphy. In 2017, the World Health Organization (WHO) updated the classifications of all gastroenteropancreatic NETs based upon tumor cell differentiation, mitotic count and proliferative index (Ki-67). The treatment strategies are conducted according to tumor size, tumor cell differentiation, proliferation, and liver metastasis or not. This review article summaries the current knowledge of GI-NET, including the epidemiology, clinical presentation, diagnosis, staging and updated treatment. Clinicians will learn more about GI-NET from this paper to increase the early diagnosis rate which leads toproper management followed by improved overall survival.