[Diagnosis and treatment of multiple endocrine neoplasia type 1 related pancreatic endocrine tumors].
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.
Yang
Jun
J
Department of Surgery, Shanghai Jiaotong University School of Medicine Affiliated Ruijin Hospital, Shanghai 200025, China.
Zhou
Guang-wen
GW
Chen
Xi
X
Wei
Yao
Y
Peng
Cheng-hong
CH
Ning
Guang
G
Li
Hong-wei
HW
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