Surgical management of concomitant pancreatic neuroendocrine tumors inhereditary tumor syndromes
10.19538/j.cjps.issn1005-2208.2019.09.10
- Author:
Xu HAN
1
;
Wen-hui LOU
1
Author Information
1. Department of General Surgery,Zhongshan Hospital,Fudan University Shanghai 200032,China
- Publication Type:Journal Article
- Keywords:
pancreatic neuroendocrine tumors;
hereditary tu-mor syndromes;
multiple endocrine neoplasia type 1;
von Hip-pel-Lindau syndrome;
tuberous sclerosis;
neurofibromatosis type 1
- From:
Chinese Journal of Practical Surgery
2019;39(09):921-925
- CountryChina
- Language:Chinese
-
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.
