A Case of von Hippel-Lindau Disease Diagnosed by the Occurrence of Pancreatic Endocrine Tumor

Yasuhiro Morita, Kijuro Takanishi, Itaru Osaka and Jun Matsumoto

Department of Surgery, Metropolitan Fuchu Hospital

A 45-year-old man who had had surgery for cerebellar hemangioblastoma at age 31 and having no family history of von Hippel-Lindau disease (VHL disease) was found in abdominal ultrasonography to have a nonfunctional pancreatic-head tumor. Following pylorus-preserving pancreaticoduodenectomy, the pancreatic tumor was diagnosed as neuroendocrine based on inmmunohistochemical staining. This case was not diagnosed as VHL disease due to absence of a family history of it. Fourteen years later, however, pancreatic tumor surgery confirmed VHL disease. Disease associated with VHL disease thus requires lomg-term follow-up to definitively confirm family VHL disease.

Key words
pancreatic endocrine tumor, von Hippel-Lindau disease, pancreatic serous cystadenoma

Reprint requests
Yasuhiro Morita Department of Surgery, Metropolitan Fuchu Hospital
2-9-2 Musashidai, Fuchu, 183-0042 Japan

Accepted
June 18, 2009

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