胰神經內分泌瘤

胰神經內分泌瘤（Pancreatic neuroendocrine tumors，簡稱PanNETs、PETs，或PNETs），是一种源自於胰臟神经分泌细胞的腫瘤。它是屬於一種神經內分泌腫瘤，可能為惡性腫瘤或良性肿瘤，其发病率约占胃腸胰神經內分泌腫瘤（GEP-NETs）的1/3。 PanNETs並不常見，僅占所有胰臟腫瘤的 1 至 2% 。 PanNET可以根據功能性，與腫瘤分級和分期來進行分類： 功能型（functional）和非功能型（nonfunctional）兩大類，功能型PanNETs的腫瘤會過度分泌激素，造成患者產生相對應的症狀，反之則為非功能型。功能型約占所有PanNETs的 10-60 % ，通常會較非功能型早期診斷，且幾乎皆為良性。 2017年世界衛生組織（WHO）分類系統將 PanNETs 分為三級。該分類系統相對於過去的分類，更強調腫瘤的分級。 目前關於PanNETs分類系統主要有歐洲內分泌腫瘤協會（ENETS）系統，以及AJCC系統兩種，兩者皆使用TNM系統進行分期。 功能型PanNETs的症狀大多與其分泌激素相關，主要症狀源自於該激素過量的表現。非功能型雖也會分泌多種激素，但其激素過量症狀並不若功能型那樣明顯。非功能型PanNETs主要的症狀源自於壓迫周圍器官或是遠端轉移的症狀。但隨著影像學的進步，非功能型PanNETs有時會在壓迫症狀出現之前，就意外診斷出來。有時時非功能型的PanNETs也會在疾病後期出現激素過量症狀。 PanNETs過去又常被稱為胰島細胞瘤（islet cell tumors），但該名稱無法準確描述源自於胰島之外的PanNETs，因此使用該名稱者已逐漸減少。 胰臟癌 神經內分泌腫瘤 Burns WR, Edil BH. Neuroendocrine pancreatic tumors: guidelines for management and update. Current treatment options in oncology. March 2012, 13 (1): 24–34. PMID 22198808. doi:10.1007/s11864-011-0172-2.  Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ) Health Professional Version. National Cancer Institute. March 7, 2014. [1] The PanNET denomination is in line with current WHO guidelines. Historically, PanNETs have also been referred to by a variety of terms, and are still often called "islet cell tumors" or "pancreatic endocrine tumors". See: Klimstra DS, Modlin IR, Coppola D, et al. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems (PDF). Pancreas. August 2010, 39 (6): 707–12 [2018-05-17]. PMID 20664470. doi:10.1097/MPA.0b013e3181ec124e. （原始内容 (PDF)存档于2015-09-24）.  Oberg, K. Pancreatic endocrine tumors. Seminars in Oncology. 2010, 37 (6): 594–618. PMID 21167379. doi:10.1053/j.seminoncol.2010.10.014.  Sun, Jian. Pancreatic neuroendocrine tumors. Intractable & Rare Diseases Research. 2017, 6 (1): 21–28. ISSN 2186-361X. doi:10.5582/irdr.2017.01007 （英语）.  Vinik, Aaron; Casellini, Carolina; Perry, Roger R.; Feliberti, Eric; Vingan, Harlan. De Groot, Leslie J.; Chrousos, George; Dungan, Kathleen; Feingold, Kenneth R.; Grossman, Ashley; Hershman, Jerome M.; Koch, Christian; Korbonits, Márta; McLachlan, Robert , 编. Endotext. South Dartmouth (MA): MDText.com, Inc. 2015 [2018-05-17]. PMID 25905300. （原始内容存档于2021-09-29）.  Klimstra, David S.; Modlin, Irvin R.; Coppola, Domenico; Lloyd, Ricardo V.; Suster, Saul. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems (PDF). Pancreas. August 2010, 39 (6): 707–712 [2018-05-17]. ISSN 1536-4828. PMID 20664470. doi:10.1097/MPA.0b013e3181ec124e. （原始内容 (PDF)存档于2015-09-24）.  Scoazec, Jean-Yves; Couvelard, Anne. Classification des tumeurs neuroendocrines pancréatiques : nouveautés introduites par la classification OMS 2017 des tumeurs des organes endocrines et perspectives. Annales de Pathologie. 2017-12, 37 (6): 444–456. ISSN 0242-6498. doi:10.1016/j.annpat.2017.10.003 （英语）.  Öberg K, Knigge U, Kwekkeboom D, Perren A. Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology. October 2012,. 23 Suppl 7: vii124–30 [2018-05-17]. PMID 22997445. doi:10.1093/annonc/mds295. （原始内容存档于2013-10-11）.  (Table 5 Archive.is的存檔，存档日期2014-12-26 outlines the proposed TNM staging system for PanNETs.) Classification, epidemiology, clinical presentation, localization, and staging of pancreatic neuroendocrine neoplasms. www.uptodate.com. UpToDate. [2018-05-17].  Vagefi, Parsia A.; Razo, Oswaldo; Deshpande, Vikram; McGrath, Deborah J.; Lauwers, Gregory Y.; Thayer, Sarah P.; Warshaw, Andrew L.; Fernández-Del Castillo, Carlos. Evolving patterns in the detection and outcomes of pancreatic neuroendocrine neoplasms: the Massachusetts General Hospital experience from 1977 to 2005. Archives of Surgery (Chicago, Ill.: 1960). 2007-4, 142 (4): 347–354. ISSN 0004-0010. PMC 3979851 . PMID 17438169. doi:10.1001/archsurg.142.4.347.  请检查|date=中的日期值 (帮助) Li, Ji; Luo, Guopei; Fu, Deliang; Jin, Chen; Hao, Sijie; Yang, Feng; Wang, Xiaoyi; Yao, Lie; Ni, Quanxing. Preoperative diagnosis of nonfunctioning pancreatic neuroendocrine tumors. Medical Oncology (Northwood, London, England). 2011-12, 28 (4): 1027–1031. ISSN 1559-131X. PMID 20623205. doi:10.1007/s12032-010-9611-3.  Nomura, Naohiro; Fujii, Tsutomu; Kanazumi, Naohito; Takeda, Shin; Nomoto, Shuji; Kasuya, Hideki; Sugimoto, Hiroyuki; Yamada, Suguru; Nakao, Akimasa. Nonfunctioning neuroendocrine pancreatic tumors: our experience and management. Journal of Hepato-Biliary-Pancreatic Surgery. 2009, 16 (5): 639–647. ISSN 1436-0691. PMID 19365596. doi:10.1007/s00534-009-0099-1.  Neuroendocrine tumors, NCCN Guidelines Version 1.2015 (PDF). NCCN Guidelines. National Comprehensive Cancer Network, Inc. November 11, 2014 [December 25, 2014]. （原始内容存档 (PDF)于2021-04-24）.  de Mestier, Louis; Hentic, Olivia; Cros, Jérôme; Walter, Thomas; Roquin, Guillaume; Brixi, Hedia; Lombard-Bohas, Catherine; Hammel, Pascal; Diebold, Marie-Danièle. Metachronous hormonal syndromes in patients with pancreatic neuroendocrine tumors: a case-series study. Annals of Internal Medicine. 2015-05-19, 162 (10): 682–689 [2018-05-17]. ISSN 1539-3704. PMID 25984844. doi:10.7326/M14-2132. （原始内容存档于2020-05-15）.  开放目录项目中的“胰神經內分泌瘤”