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。
胰神經內分泌腫瘤 | |
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同义词 | 胰島細胞瘤(islet cell tumors)[1][2],或胰內分泌細胞瘤(pancreaticdocrine tumors)[3][4] |
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胰臟示意圖 | |
类型 | islet cell tumor[*]、gastrointestinal carcinoma[*]、疾病 |
分类和外部资源 | |
醫學專科 | 肿瘤学 |
ICD-11 | XH5XB7 |
ICD-10 | C25.4 |
OMIM | [2] |
Orphanet | 97253 |
PanNETs並不常見,僅占所有胰臟腫瘤的 1 至 2% 。
分類
PanNET可以根據功能性,與腫瘤分級和分期來進行分類:
功能性分類
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[5]。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[5]。
類型 | 腫瘤原發位置 | 生物標記 | 症狀 | 備註 |
---|---|---|---|---|
胰島素瘤(Insulinoma) | 胰臟頭部、體部,及尾部 | 胰島素、胰島素原、C-胜肽(C-peptide) | 低血糖、惠普三要項(Whipple triad) | 最常見的PanNETs |
胃泌素瘤(Gastrinoma) | 胃泌素瘤三角(Gastrinoma triangle) | 胃泌素瘤、 胰多肽(polypeptide,PP) | Zollinger–Ellison症候群(胃潰瘍、上腹痛、腹瀉) | 次常見 |
血管活性腸肽瘤(VIPoma) | 胰臟體部,及尾部 | 血管活性腸肽(VIP) | Verner-Morrison症候群(水瀉、低血鉀、脫水、胃酸缺乏) | 罕見 |
升糖素瘤(Glucagonoma) | 胰臟體部,及尾部 | 升糖素、腸升糖素(glycentin) | 紅疹、遊走性紅斑、糖尿病、惡病質 | 罕見 |
體制素瘤(Somatostatinoma) | 胰十二指腸溝(Pancreatoduodenal groove)、壺腹、壺腹周圍 | 體抑素 | 糖尿病、膽石病、腹瀉 | 罕見 |
胰多肽瘤(PPoma) | 胰臟頭部 | 胰多肽 | 罕見 | |
促腎上腺皮質素瘤(ACTHoma) | 胰臟 | 促腎上腺皮質激素(ACTH) | 庫欣氏症候群 | 罕見,發生於胰臟者占異位性庫欣氏症的4-16% |
類癌 | 胰臟 | 血清素 | 潮紅、腹瀉 | 罕見,發生於胰臟者占所有類癌症後群的1% |
副甲狀腺荷爾蒙相關蛋白瘤(PTHrp-oma) | 胰臟 | 副甲狀腺荷爾蒙相關蛋白(PTHrp) | 高血鈣症狀,症狀會類似副甲狀腺過高 | 罕見 |
腫瘤分級
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[7]。
分類/分級 | Ki-67增殖指數*(%) | 有絲分裂指數(%) |
---|---|---|
分化良好的PanNETs | ||
PanNET G1 | <3 | <2 |
PanNET G2 | 3 to 20 | 2 to 20 |
PanNET G3 | >20 | >20 |
分化不好的PanNETs:胰臟神經內分泌癌(Pancreatic neuroendocrine carcinomas,PanNECs) | ||
PanNEC (G3) | >20 | >20 |
小細胞型(Small cell type) | ||
大細胞型(Large cell type) | ||
混合型:腫瘤同時由神經內分泌細胞及非神經內分泌細胞構成 |
腫瘤分期
目前關於PanNETs分類系統主要有歐洲內分泌腫瘤協會(ENETS)系統[9],以及AJCC系統兩種,兩者皆使用TNM系統進行分期[5]。
症狀及徵象
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[10]。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[11][12][13]。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[14]:43–44。有時時非功能型的PanNETs也會在疾病後期出現激素過量症狀[15]。
命名
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[10]。
參見
參考文獻
- ^ 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.
- ^ 5.0 5.1 5.2 5.3 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.)
- ^ 10.0 10.1 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.
- ^ 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).