Multiple endocrine neoplasia type 1 simultaneous with various types of enteropancreatic endocrine tumors: one case report: lessons learned.
Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disorder characterized by the development of parathyroid hyperplasia, pancreatic endocrine tumors, pituitary adenomas, and adrenal adenomas. We reported 1 case of MEN 1 simultaneous with gastrinoma and insulinoma; meanwhile, insulinomas were ectopic and recurrent. The genetic screening showed the mutation of 427del AT of the MEN 1 gene. Surgical removal is considered the treatment of choice, with limited adverse effects and relatively low morbidity and mortality. She was treated by means of several surgical strategies, resulting in improvement of the frequency and severity of the hypoglycemic episodes and a better quality of life.
Zhou
Guangwen
G
Department of Surgery, RuijinHospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
Wei
Yao
Y
Chen
Xi
X
Jiang
Xiaohua
X
Gu
Weiqiong
W
Li
Xiaoying
X
Peng
Chenhong
C
Ning
Guang
G
Li
Hongwei
H
D000328:Adult; D005260:Female; D015408:Gastrinoma / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007340:Insulinoma / Q000235:genetics / Q000601:surgery*; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics / Q000601:surgery*; D009154:Mutation; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000601:surgery*; D010282:Parathyroid Neoplasms / Q000235:genetics / Q000601:surgery*; D010911:Pituitary Neoplasms / Q000235:genetics / Q000601:surgery*; D011518:Proto-Oncogene Proteins / Q000235:genetics; D011788:Quality of Life; D016896:Treatment Outcome
C105373:MEN1 protein, human; D011518:Proto-Oncogene Proteins