Zhang Ming-Yi, He Du, Zhang Shuang
Department of Biotherapy, State Key Laboratory of Biotherapy and Cancer Center, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
World J Gastrointest Oncol. 2020 Jul 15;12(7):705-718. doi: 10.4251/wjgo.v12.i7.705.
In 2017 the World Health Organization revised the criteria for classification of pancreatic neuroendocrine neoplasms (pNENs) after a consensus conference at the International Agency for Research on Cancer. The major change in the new classification was to subclassify the original G3 group into well-differentiated pancreatic neuroendocrine tumors G3 (pNETs G3) and poorly differentiated pancreatic neuroendocrine carcinomas (pNECs), which have been gradually proven to be completely different in biological behavior and clinical manifestations in recent years. In 2019 this major change subsequently extended to NENs involving the entire digestive tract. The updated version of the pNENs grading system marks a growing awareness of these heterogeneous tumors. This review discusses the clinicopathological, genetic and therapeutic features of poorly differentiated pNECs and compare them to those of well-differentiated pNETs G3. For pNETs G3 and pNECs (due to their lower incidence), there are still many problems to be investigated. Previous studies under the new grading classification also need to be reinterpreted. This review summarizes the relevant literature from the perspective of the differences between pNETs G3 and pNECs in order to deepen understanding of these diseases and discuss future research directions.
2017年，世界卫生组织在国际癌症研究机构召开共识会议后，修订了胰腺神经内分泌肿瘤（pNENs）的分类标准。新分类中的主要变化是将原来的G3组细分为高分化胰腺神经内分泌肿瘤G3（pNETs G3）和低分化胰腺神经内分泌癌（pNECs），近年来已逐渐证明它们在生物学行为和临床表现上完全不同。2019年，这一主要变化随后扩展到累及整个消化道的神经内分泌肿瘤。pNENs分级系统的更新版本标志着对这些异质性肿瘤的认识不断提高。本综述讨论了低分化pNECs的临床病理、遗传和治疗特征，并将它们与高分化pNETs G3的特征进行比较。对于pNETs G3和pNECs（由于其发病率较低），仍有许多问题有待研究。新分级分类下的既往研究也需要重新解读。本综述从pNETs G3和pNECs的差异角度总结相关文献，以加深对这些疾病的理解并讨论未来的研究方向。
