Zhonghua wai ke za zhi [Chinese journal of surgery] · 2009
[Diagnosis and treatment of multiple endocrine neoplasia type 1 related pancreatic endocrine tumors].
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Yang Jun, Zhou Guang-wen, Chen Xi, Wei Yao, Peng Cheng-hong, Ning Guang, Li Hong-wei
Аннотация
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.
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.