Yang Jun, Zhou Guang-wen, Chen Xi, Wei Yao, Peng Cheng-hong, Ning Guang, Li Hong-wei
Department of Surgery, Shanghai Jiaotong University School of Medicine Affiliated Ruijin Hospital, Shanghai 200025, China.
Zhonghua Wai Ke Za Zhi. 2009 Mar 1;47(5):329-32.
To summarize the experience on diagnosis and treatment of multiple endocrine neoplasia type 1 (MEN-1) related pancreatic endocrine tumors (PET).
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.
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.
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.
总结1型多发性内分泌腺瘤病（MEN-1）相关胰腺内分泌肿瘤（PET）的诊治经验。
2004年1月至2007年12月，上海交通大学医学院附属瑞金医院收治10例MEN-1相关PET患者。其中男性2例，女性8例，年龄11～49岁。通过实验室检查、影像学检查及基因测序进行诊断，并对患者采用药物治疗、手术及随访。
9例为胰岛素瘤，其中2例为多发胰岛素瘤，1例为单发胰岛素瘤、多发无功能PET及伴肝转移的恶性十二指肠胃泌素瘤；另1例临床诊断为胰高血糖素瘤。5例有家族史，9例检测到MEN-1基因突变。7例患者接受了9次手术。手术中发现12个胰岛素瘤、4个无功能PET及1个十二指肠胃泌素瘤。所有患者随访1个月至11年，9例情况良好，1例死亡。
充分认识PET及MEN-1，早期诊断MEN-1相关PET，适当的手术干预可改善患者生活质量，延长患者生存时间。
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