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【消化器全般】MiNEN:混合型神経内分泌・非神経内分泌腫瘍レビュー(+α)
Digestive System Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms
Neuroendocrinology 2017;105:412–425 (Free article)
Abstract
Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) are a heterogeneous subgroup of rare neoplasms that represent about a third of all poorly differentiated neuroendocrine carcinomas (PDNEC). MiNEN combine a neuroendocrine component, usually a PDNEC, and a non-neuroendocrine component, generally an adenocarcinoma, both accounting for at least 30% of the neoplasm. MiNEN are classified as high-, intermediate-, or low-grade malignancies depending on the metastatic potential of the tumour components. High-grade malignant component should be considered even if it represents <30% of the tumour. The prognosis of MiNEN is generally intermediate between those of the two "pure" components composing it. The aim of this comprehensive review of the literature is to suggest a standardized management of MiNEN. An increasing body of evidence suggests that PDNEC components share molecular abnormalities with their adenocarcinoma counterparts, but also display additional alterations. This advocates for a common origin, and that the presence of a PDNEC component in an adenocarcinoma could indicate a turning point in carcinogenesis.
Keywords: Adenocarcinoma; Chemotherapy; Epidemiology; Mixed tumours; Neuroendocrine tumours; Prognosis; Treatment.
【SANOTIC SUMMARY】本論文の内容+α
1.NEN は高分化型と低分化型があり、両者は全く異なる
👉高分化 NEN=NET:いわゆるカルチノイドの形態
SSTR の発現が診断・治療ともに有用
👉低分化 NEN=NEC:小細胞癌・大細胞神経内分泌癌
p53、Rb の異常が多い
2.増殖能(MIB-1、核分裂数)で Grading する
👉MIB-1/Ki-67 に関して
NET G1(~2%)、NET G2(2~20%)、NET G3(20%~)
NEC は通常 20% 以上(G3)に分類されるが、通常 55% 以上
※ NET G3 と NEC の鑑別では、①形態、②遺伝子・蛋白(p53, Rb, SSTR など)のほかに、③MIB-1/Ki-67 の陽性率も有用である。
3.MiNENは神経内分泌成分・非神経内分泌成分の両方が 30% 以上
👉両成分は組織型を問わない
腺癌+NEC(MANEC:mixed adenoneuroendocrine carcinoma)が多い
👉NEC 成分は 30% 未満でも予後不良なので診断に付記すべし
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