Abstract:

Surgical management of concomitant pancreatic neuroendocrine tumors inhereditary tumor syndromes              HAN Xu，LOU Wen-hui. Department of General Surgery, Zhongshan Hospital, Fudan University，Shanghai200032，China
Corresponding author: LOU Wen-hui, E-mail：lou.wenhui
@zs-hospital.sh.cn
Abstract    Pancreatic neuroendocrine neoplasms（pNENs） might present as part of complex hereditary （familial） syndromes caused by germline mutations such as multiple endocrine neoplasia type 1 （MEN1），von Hippel-Lindau disease（VHL），tuberous sclerosis，and neurofibromatosis type 1 （NF1）.Increasing knowledge of hereditary pNENs in both their genetic underpinnings and clinical implications has affected the entire spectrum of their surgical management. However，the appropriate surgical timing and management of concomitant pNENs inhereditary tumor syndromes is still debated.

Key words: pancreatic neuroendocrine tumors, hereditary tumor syndromes, multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, tuberous sclerosis, neurofibromatosis type 1

摘要：

尽管多数胰腺神经内分泌肿瘤（pNENs）为散发，但其中一部分是由胚系突变导致的遗传性肿瘤综合征，多发性内分泌肿瘤综合征1型（MEN-1）是胰腺神经内分泌肿瘤病人最常合并的遗传性肿瘤综合征，其次是林岛综合征（VHL病），结节性硬化症和神经纤维瘤病1型（NF1），合并遗传性肿瘤综合征的pNENs异质性和复杂性导致其诊断较为困难，该类疾病的诊治和随访应由拥有pNENs诊治经验的多学科综合治疗协作组（MDT）进行管理。遗传性肿瘤综合征中的pNENs病灶存在恶变风险，其手术介入的正确时机和切除范围一直存在争议。

关键词: 胰腺神经内分泌肿瘤, 遗传性肿瘤综合征, 多发性内分泌肿瘤综合征1型, 林岛综合征, 结节性硬化症, 神经纤维瘤病1型