Crippa Stefano, Partelli Stefano, Belfiori Giulio, Palucci Marco, Muffatti Francesca, Adamenko Olga, Cardinali Luca, Doglioni Claudio, Zamboni Giuseppe, Falconi Massimo
Stefano Crippa, Stefano Partelli, Marco Palucci, Francesca Muffatti, Olga Adamenko, Massimo Falconi, Division of Pancreatic Surgery, Pancreas Translational and Clinical Research Center, San Raffaele Scientific Institute, Vita e Salute University, 20132 Milan, Italy.
World J Gastroenterol. 2016 Dec 7;22(45):9944-9953. doi: 10.3748/wjg.v22.i45.9944.
Neuroendocrine carcinomas (NEC) of the pancreas are defined by a mitotic count > 20 mitoses/10 high power fields and/or Ki67 index > 20%, and included all the tumors previously classified as poorly differentiated endocrine carcinomas. These latter are aggressive malignancies with a high propensity for distant metastases and poor prognosis, and they can be further divided into small- and large-cell subtypes. However in the NEC category are included also neuroendocrine tumors with a well differentiated morphology but ki67 index > 20%. This category is associated with better prognosis and does not significantly respond to cisplatin-based chemotherapy, which represents the gold standard therapeutic approach for poorly differentiated NEC. In this review, the differences between well differentiated and poorly differentiated NEC are discussed considering both pathology, imaging features, treatment and prognostic implications. Diagnostic and therapeutic flowcharts are proposed. The need for a revision of current classification system is stressed being well differentiated NEC a more indolent disease compared to poorly differentiated tumors.
胰腺神经内分泌癌（NEC）的定义为有丝分裂计数>20个有丝分裂/10个高倍视野和/或Ki67指数>20%，并包括所有先前分类为低分化内分泌癌的肿瘤。后者是侵袭性恶性肿瘤，远处转移倾向高且预后差，可进一步分为小细胞和大细胞亚型。然而，NEC类别中还包括形态学上分化良好但Ki67指数>20%的神经内分泌肿瘤。这一类别预后较好，对以顺铂为基础的化疗无明显反应，而顺铂化疗是低分化NEC的金标准治疗方法。在本综述中，从病理学、影像学特征、治疗及预后影响等方面讨论了高分化和低分化NEC之间的差异。提出了诊断和治疗流程图。强调了修订当前分类系统的必要性，因为与低分化肿瘤相比，高分化NEC是一种惰性更强的疾病。
