Division of Hematology and Medical Oncology, UH Seidman Cancer Center, Case Comprehensive Cancer Center, Case Western Reserve University, Cleveland, Ohio, USA.
Department of Medicine, UH Seidman Cancer Center Case Comprehensive Cancer Center, Case Western Reserve University, Cleveland, Ohio, USA.
Endocr Relat Cancer. 2024 Sep 21;31(10). doi: 10.1530/ERC-24-0025. Print 2024 Oct 1.
The classification and management of neuroendocrine neoplasms (NENs) arising in the tubular gastrointestinal (GI) tract and pancreas have significantly evolved over the last decades. In the latest WHO classification published in 2022, NENs are separated regardless of their primary origin into two main groups: well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The substantial changes in the grading system changed the definition of grade 3 to include high-grade well-differentiated NETs (G3-NETs), and poorly differentiated NECs (-NECs). Although these two subgroups are considered high grades with Ki-67 >20%, they have different genomic profiles, prognosis, and clinical behavior, which critically influence their treatment strategies. The available clinical trial data to guide therapy of these high-grade subgroups are extremely limited, which impacts their management. In this review, we will summarize the current advances in the multidisciplinary approach for the management of high-grade gastroenteropancreatic NENs (GEP-NENs) including G3-NETs and NECs.
在过去的几十年中，起源于管状胃肠道和胰腺的神经内分泌肿瘤（NENs）的分类和管理有了显著的发展。在 2022 年发布的最新版世界卫生组织分类中，无论其原发部位如何，NENs 均分为两个主要组：分化良好的神经内分泌肿瘤（NETs）和分化差的神经内分泌癌（NECs）。分级系统的重大变化改变了 3 级的定义，包括高级别分化良好的 NET（G3-NET）和分化差的 NEC（-NEC）。尽管这两个亚组被认为是 Ki-67＞20%的高级别肿瘤，但它们具有不同的基因组特征、预后和临床行为，这对其治疗策略有重大影响。指导这些高级别亚组治疗的可用临床试验数据极为有限，这影响了它们的管理。在这篇综述中，我们将总结高级别胃肠胰神经内分泌肿瘤（GEP-NENs），包括 G3-NET 和 NEC 的多学科治疗方法的最新进展。
