A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1

World Journal of Surgical Oncology

Yoshitsugu Tajima¹  Riruke Maruyama²  Seiji Yano¹  Noriyoshi Ishikawa²  Hiroshi Imaoka³  Youko Hari¹  Yasunari Kawabata¹  Takeshi Nishi¹ 

[1] Department of Digestive and General Surgery, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, 693-8501, Shimane, Japan;Department of Organ Pathology, Shimane University Faculty of Medicine, Shimane, Japan;Department of Gastroenterology and Hepatology, Shimane University Faculty of Medicine, Shimane, Japan

关键词: von Recklinghausen’s disease;    Pancreatic neuroendocrine tumor;    Pancreatic acinar-endocrine carcinoma;    Neurofibromin;    Neurofibromatosis-1;    Neuroendocrine tumor;   

Others  :  827463
DOI  :  10.1186/1477-7819-10-153

 received in 2012-03-21, accepted in 2012-07-06,  发布年份 2012

【 摘 要 】

Patients with neurofibromatosis-1 (NF-1) sometime develop neuroendocrine tumors (NET). Although these NETs usually occur in the duodenum or peri-ampullary region, they occasionally grow in the pancreas (PNET). A 62-year-old man with NF-1 had mild liver dysfunction and was admitted to our hospital for further examination. An abdominal contrast-enhanced computed tomography scan demonstrated a 30-mm tumor in the head of the pancreas. The scan showed an invasion of the tumor into the duodenum, and biopsy under an endoscopic ultrasonography indicated that the tumor was a NET. A subtotal stomach-preserving pancreaticoduodenectomy was performed. Macroscopically, the pancreatic tumor was white and elastic hard. Microscopically, tumor cells were composed of ribbons, cords, and solid nests with an acinus-like structure. The tumor was diagnosed as NET G2 according to the WHO classification (2010). The product of theNF-1 gene, i.e., neurofibromin, was weakly positive in the tumor cells, suggesting that the tumor was induced by a mutation in the NF-1 gene. This is the seventh case of PNET arising in NF-1 patients worldwide.

【 授权许可】

   
2012 Nishi et al.; licensee BioMed Central Ltd.

【 图 表 】

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