Feola Tiziana, Centello Roberta, Sesti Franz, Puliani Giulia, Verrico Monica, Di Vito Valentina, Di Gioia Cira, Bagni Oreste, Lenzi Andrea, Isidori Andrea M, Giannetta Elisa, Faggiano Antongiulio
Department of Experimental Medicine, "Sapienza" University of Rome, 00161 Rome, Italy.
Neuroendocrinology, Neuromed Institute, IRCCS, 86077 Pozzilli (IS), Italy.
Cancers (Basel). 2021 Mar 12;13(6):1247. doi: 10.3390/cancers13061247.
Highly proliferative (G3) neuroendocrine neoplasms are divided into well differentiated tumors (NETs) and poorly differentiated carcinomas (NECs), based on the morphological appearance. This systematic review aims to evaluate the clinicopathological features and the treatment response of the NEC subgroup with a Ki67 labeling index (LI) < 55%.
A literature search was performed using MEDLINE, Cochrane Library, and Scopus between December 2019 and April 2020, last update in October 2020. We included studies reporting data on the clinicopathological characteristics, survival, and/or therapy efficacy of patients with NECs, in which the Ki67 LI was specified.
8 papers were included, on a total of 268 NEC affected patients. NECs with a Ki67 LI < 55% have been reported in patients of both sexes, mainly of sixth decade, pancreatic origin, and large-cell morphology. The prevalent treatment choice was chemotherapy, followed by surgery and, in only one study, peptide receptor radionuclide therapy. The subgroup of patients with NEC with a Ki67 LI < 55% showed longer overall survival and progression free survival and higher response rates than the subgroup of patients with a tumor with higher Ki67 LI (≥55%).
NECs are heterogeneous tumors. The subgroup with a Ki67 LI < 55% has a better prognosis and should be treated and monitored differently from NECs with a Ki67 LI ≥ 55%.
高度增殖性（G3）神经内分泌肿瘤根据形态学表现分为高分化肿瘤（NETs）和低分化癌（NECs）。本系统评价旨在评估Ki67标记指数（LI）<55%的NEC亚组的临床病理特征和治疗反应。
于2019年12月至2020年4月使用MEDLINE、Cochrane图书馆和Scopus进行文献检索，最后一次更新于2020年10月。我们纳入了报告NEC患者临床病理特征、生存情况和/或治疗疗效数据的研究，其中明确了Ki67 LI。
纳入8篇论文，共268例NEC患者。Ki67 LI<55%的NEC在男女患者中均有报道，主要为60岁左右，起源于胰腺，具有大细胞形态。最常用的治疗选择是化疗，其次是手术，仅在一项研究中采用了肽受体放射性核素治疗。Ki67 LI<55%的NEC患者亚组的总生存期和无进展生存期更长，缓解率高于Ki67 LI较高（≥55%）的肿瘤患者亚组。
NEC是异质性肿瘤。Ki67 LI<55%的亚组预后较好，其治疗和监测应与Ki67 LI≥55%的NEC不同。
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