Pellat Anna, Coriat Romain
Department of Gastroenterology and digestive oncology, Cochin Teaching Hospital, AP-HP, 75014 Paris, France.
Faculté de Médecine, Université de Paris, 75006 Paris, France.
J Clin Med. 2020 Jun 1;9(6):1677. doi: 10.3390/jcm9061677.
The 2017 World Health Organization (WHO) classification of neuroendocrine neoplasms (NEN) of the digestive tract introduced a new category of tumors named well-differentiated grade 3 neuroendocrine tumors (NET G-3). These lesions show a number of mitosis, or a Ki-67 index higher than 20% with a well-differentiated morphology, therefore separating them from neuroendocrine carcinomas (NEC) which are poorly differentiated. It has become clear that NET G-3 show differences not only in morphology but also in genotype, clinical presentation, and treatment response. The incidence of digestive NET G-3 represents about one third of NEN G-3 with main tumor sites being the pancreas, the stomach and the colon. Treatment for NET G-3 is not yet standardized because of lack of data. In a non-metastatic setting, international guidelines recommend surgical resection, regardless of tumor grading. For metastatic lesion, chemotherapy is the main treatment with similar regimen as NET G-2. Sunitinib has also shown some positive results in a small sample of patients but this needs confirmation. Peptide receptor radionuclide therapy (PRRT) and immunotherapy could be future available treatments after ongoing studies. The goal of this review was to sum up the latest data on the epidemiology and management of digestive NET G-3.
2017年世界卫生组织（WHO）发布的消化道神经内分泌肿瘤（NEN）分类引入了一类新的肿瘤，即高分化3级神经内分泌肿瘤（NET G-3）。这些病变具有一定数量的核分裂象，或Ki-67指数高于20%，且形态学表现为高分化，因此与低分化的神经内分泌癌（NEC）相区分。现已明确，NET G-3不仅在形态学上，而且在基因型、临床表现和治疗反应方面均存在差异。消化道NET G-3的发病率约占NEN G-3的三分之一，主要肿瘤部位为胰腺、胃和结肠。由于缺乏数据，NET G-3的治疗尚未标准化。在非转移情况下，国际指南推荐手术切除，无论肿瘤分级如何。对于转移性病变，化疗是主要治疗方法，方案与NET G-2相似。舒尼替尼在一小部分患者中也显示出一些阳性结果，但这需要进一步证实。正在进行的研究表明，肽受体放射性核素治疗（PRRT）和免疫治疗可能成为未来可用的治疗方法。本综述的目的是总结消化道NET G-3的流行病学和管理方面的最新数据。
