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 | |
---|---|
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-10 | C25.4 |
OMIM | |
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 |
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]。
參考文獻
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