Department of Medical Oncology, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium.
Curr Opin Oncol. 2021 Jul 1;33(4):378-385. doi: 10.1097/CCO.0000000000000740.
Neuroendocrine neoplasms (NENs) are rare and heterogeneous malignancies whose natural evolution may be defined according to various prognostic factors, including localization of the primitive tumour, hormone secretory status, histological grade, tumour burden, tumour growth rate, expression of somatostatin receptors and fluorodeoxyglucose-avidity. The treatment of these tumours in an advanced setting is based on relatively little robust data.
A recent pathological classification introduced a new category of high-grade but well differentiated neuroendocrine tumours (NET G3), with markedly different behaviour from neuroendocrine carcinomas (NECs). Yet, the optimal treatment of those tumours is still uncertain. Advances are needed in molecular subtyping of NENs to understand better their heterogeneity and inform personalized therapies.
The current review summarizes the current knowledge, indicates some exciting future directions and outlines the most interesting ongoing clinical trials likely to impact current practice.
神经内分泌肿瘤（NENs）是罕见的异质性恶性肿瘤，其自然病程可根据多种预后因素来定义，包括原始肿瘤的定位、激素分泌状态、组织学分级、肿瘤负荷、肿瘤生长速度、生长抑素受体表达和氟脱氧葡萄糖摄取。晚期这些肿瘤的治疗主要基于相对较少的稳健数据。
最近的病理分类引入了一个新的高级别但分化良好的神经内分泌肿瘤（NET G3）类别，其行为与神经内分泌癌（NEC）明显不同。然而，这些肿瘤的最佳治疗方法仍不确定。需要在 NENs 的分子亚型方面取得进展，以便更好地了解其异质性并为个性化治疗提供信息。
本文综述了目前的知识，指出了一些令人兴奋的未来方向，并概述了最有趣的正在进行的临床试验，这些试验可能会影响当前的实践。
