Surgical management of concomitant pancreatic neuroendocrine tumors inhereditary tumor syndromes / 中国实用外科杂志

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 type1(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.