Pellat Anna, Cottereau Anne Ségolène, Palmieri Lola-Jade, Soyer Philippe, Marchese Ugo, Brezault Catherine, Coriat Romain
Department of Gastroenterology and Digestive Oncology, Hôpital Cochin, AP-HP, 27 rue du Faubourg Saint Jacques, Université de Paris, 75014 Paris, France.
Department of Nuclear Medicine, Hôpital Cochin, AP-HP, 27 rue du Faubourg Saint Jacques, Université de Paris, 75014 Paris, France.
Cancers (Basel). 2021 May 18;13(10):2448. doi: 10.3390/cancers13102448.
Digestive well-differentiated grade 3 neuroendocrine tumors (NET G-3) have been clearly defined since the 2017 World Health Organization classification. They are still a rare category lacking specific data and standardized management. Their distinction from other types of neuroendocrine neoplasms (NEN) not only lies in morphology but also in genotype, aggressiveness, functional imaging uptake, and treatment response. Most of the available data comes from pancreatic series, which is the most frequent tumor site for this entity. In the non-metastatic setting, surgical resection is recommended, irrespective of grade and tumor site. For metastatic NET G-3, chemotherapy is the main first-line treatment with temozolomide-based regimen showing more efficacy than platinum-based regimen, especially when Ki-67 index <55%. Targeted therapies, such as sunitinib and everolimus, have also shown some positive therapeutic efficacy in small samples of patients. Functional imaging plays a key role for detection but also treatment selection. In the second or further-line setting, peptide receptor radionuclide therapy has shown promising response rates in high-grade NEN. Finally, immunotherapy is currently investigated as a new therapeutic approach with trials still ongoing. More data will come with future work now focusing on this specific subgroup. The aim of this review is to summarize the current data on digestive NET G-3 and explore future directions for their management.
自2017年世界卫生组织分类以来，消化系统高分化3级神经内分泌肿瘤（NET G-3）已得到明确界定。它们仍然是一个罕见的类别，缺乏具体数据和标准化管理。它们与其他类型的神经内分泌肿瘤（NEN）的区别不仅在于形态，还在于基因型、侵袭性、功能成像摄取和治疗反应。现有的大多数数据来自胰腺系列，这是该实体最常见的肿瘤部位。在非转移性情况下，无论肿瘤分级和部位如何，均建议手术切除。对于转移性NET G-3，化疗是主要的一线治疗方法，基于替莫唑胺的方案比基于铂的方案显示出更高的疗效，尤其是当Ki-67指数<55%时。靶向治疗，如舒尼替尼和依维莫司，在小样本患者中也显示出一些积极的治疗效果。功能成像在检测和治疗选择中都起着关键作用。在二线或后续治疗中，肽受体放射性核素治疗在高级别NEN中显示出有希望的缓解率。最后，免疫疗法目前正在作为一种新的治疗方法进行研究，试验仍在进行中目前正在研究免疫疗法作为一种新的治疗方法，试验仍在进行中。随着未来工作更多地关注这个特定亚组，将会有更多数据出现。本综述的目的是总结目前关于消化系统NET G-3的数据，并探索其管理的未来方向。
