title: [Diagnosis and treatment of multiple endocrine neoplasia type 1 related pancreatic endocrine tumors].
abstract: OBJECTIVE
  To summarize the experience on diagnosis and treatment of multiple endocrine neoplasia type 1 (MEN-1) related pancreatic endocrine tumors (PET).
  
  
  METHODS
  From January 2004 to December 2007, there were 10 patients of MEN-1 related PET were treated in Shanghai Jiaotong University School of Medicine Affiliated Ruijin Hospital. There were 2 males and 8 females, aged from 11- to 49-years-old. They were diagnosed by laboratory tests, imaging examinations and genes sequencing. Drug therapy, surgery and follow-up were applied on the patients.
  
  
  RESULTS
  There were 9 patients having insulinomas including 2 cases of multiple insulinomas and 1 case presenting an insulinoma, multiple nonfunctional PET and malignant duodenum gastrinoma with liver metastasis. The other patient was diagnosed as glucagonoma clinically. Five cases had family history and MEN-1 gene mutation was detected in 9 patients. Seven patients accepted nine operations. Twelve insulinomas, four nonfunctional PET and one duodenum gastrinoma were found in the operations. All patients were followed up from 1 month to 11 years, and 9 patients with good conditions and 1 patient died.
  
  
  CONCLUSIONS
  Well recognizing PET and MEN-1, early diagnosing MEN-1 related PET, appropriately surgical intervention will prove patients' life quality and will help for prolonging patients' survival time.
authors: Department of Surgery, Shanghai Jiaotong University School of Medicine Affiliated Ruijin Hospital, Shanghai 200025, China.
authors: Jun
authors: J
authors: Yang
authors: Guang-wen
authors: GW
authors: Zhou
authors: Xi
authors: X
authors: Chen
authors: Yao
authors: Y
authors: Wei
authors: Cheng-hong
authors: CH
authors: Peng
authors: Guang
authors: G
authors: Ning
authors: Hong-wei
authors: HW
authors: Li
mesh_terms: D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis* / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D012189:Retrospective Studies