Does the WHO 2010 classification of pancreatic neuroendocrine neoplasms accurately characterize pancreatic neuroendocrine carcinomas?
Does the WHO 2010 classification of pancreatic neuroendocrine neoplasms accurately characterize pancreatic neuroendocrine carcinomas?
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DOI：
10.1007/s00535-014-0987-2
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发表时间：
2015-05
影响因子：
6.3
通讯作者：
Yamao K
中科院分区：
文献类型：
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作者：
Hijioka S;Hosoda W;Mizuno N;Hara K;Imaoka H;Bhatia V;Mekky MA;Tajika M;Tanaka T;Ishihara M;Yogi T;Tsutumi H;Fujiyoshi T;Sato T;Hieda N;Yoshida T;Okuno N;Shimizu Y;Yatabe Y;Niwa Y;Yamao K
根据Ki67标记指数(LI)，世卫组织在2010年将胰腺神经内分泌肿瘤归类为G1、G2和神经内分泌癌(NEC)。然而，NEC的临床行为仍未得到充分的研究。我们的目的是阐明NECS的临床病理和分子特征。我们根据世界卫生组织2010年的数据，对2001年至2014年间确诊的11例PNEC患者的临床病理特征、KRAS突变状态、治疗反应和总体生存情况进行了回顾性评估。我们将WHO-NEC细分为高分化NEC(WDNEC)和低分化NEC(PDNEC)。后者又分为大细胞亚型和小细胞亚型。Ki67 LI中位数为69.1%(范围40-95%)。11个WHO-NECs分为4个WDNEC和7个PDNEC。后者进一步分为3个大细胞亚型和4个小细胞亚型。WDNEC与PDNEC的CT血管密度分别为50%(2/4)和0%(0/7)(P=0.109)，Ki67 LI中位数分别为46.3%(40-53%)和85%(54-95%)(P=0.001)，Rb免疫阳性分别为100%(4/4)和14%(1/7)(P=0.015)；KRAS突变，0%(0/4)比86%(6/7)(P=0.015)；铂类化疗有效率0%(0/2)比100%(4/4)(P=0.067)，中位生存期227天比186天(P=0.227)。WHO-NEC类别可由不同的疾病实体组成，即WDNEC和PDNEC。这些亚群往往表现出不同的Ki67LI、Rb免疫阳性和KRAS突变，以及对化疗的不同反应。有必要对重新评估世卫组织2010年现行分类进行进一步研究。本文的在线版本(doi：10.1007/s00535-0140987-2)包含补充材料，授权用户可以使用。
The WHO classified pancreatic neuroendocrine neoplasms in 2010 as G1, G2, and neuroendocrine carcinoma (NEC), according to the Ki67 labeling index (LI). However, the clinical behavior of NEC is still not fully studied. We aimed to clarify the clinicopathological and molecular characteristics of NECs. We retrospectively evaluated the clinicopathological characteristics, KRAS mutation status, treatment response, and the overall survival of eleven pNEC patients diagnosed between 2001 and 2014 according to the WHO 2010. We subclassified WHO-NECs into well-differentiated NEC (WDNEC) and poorly differentiated NEC (PDNEC). The latter was further subdivided into large-cell and small-cell subtypes. The median Ki67 LI was 69.1 % (range 40–95 %). Eleven WHO-NECs were subclassified into 4 WDNECs and 7 PDNECs. The latter was further separated into 3 large-cell and 4 small-cell subtypes. Comparisons of WDNEC vs. PDNEC revealed the following traits: hypervascularity on CT, 50 % (2/4) vs. 0 % (0/7) (P = 0.109); median Ki67 LI, 46.3 % (40–53 %) vs. 85 % (54–95 %) (P = 0.001); Rb immunopositivity, 100 % (4/4) vs. 14 % (1/7) (P = 0.015); KRAS mutations, 0 % (0/4) vs. 86 % (6/7) (P = 0.015); response rates to platinum-based chemotherapy, 0 % (0/2) vs. 100 % (4/4) (P = 0.067), and median survival, 227 vs. 186 days (P = 0.227). The WHO-NEC category may be composed of heterogeneous disease entities, namely WDNEC and PDNEC. These subgroups tended to exhibit differing profiles of Ki67 LI, Rb immunopositivity and KRAS mutation, and distinct response to chemotherapy. Further studies for the reevaluation of the current WHO 2010 classification are warranted. The online version of this article (doi:10.1007/s00535-014-0987-2) contains supplementary material, which is available to authorized users.