胰神經內分泌瘤

胰神經內分泌瘤(Pancreatic neuroendocrine tumors,簡稱PanNETsPETs,或PNETs),是一种源自於胰臟神经分泌细胞腫瘤。它是屬於一種神經內分泌腫瘤,可能為惡性腫瘤良性肿瘤,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
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]
胰臟示意圖
类型islet cell tumor[*], gastrointestinal carcinoma[*]
分类和外部资源
醫學專科肿瘤学
ICD-10C25.4
OMIM
Orphanet97253

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]

PNET的功能性分類[6][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]

2017年WHO胰臟神經內分泌腫瘤(PanNETs)分類系統[8]
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

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