Nippon Shokakibyo Gakkai Zasshi
Online ISSN : 1349-7693
Print ISSN : 0446-6586
Case Report
Two cases of pancreatic tumor with von Hippel-Lindau disease
Hidenori KARASAKIAkira ISHIZAKINobuyuki YANAGAWAYasuhiro NAKANOJyunpei SASAJIMAYusuke MIZUKAMISatoshi TANNOYoshihiko TOKUSASHINaoyuki MIYOKAWAMitsuhiro OBARAJunichi GOTOShuichi KINOToru KONOShinichi KASAI
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2008 Volume 105 Issue 5 Pages 725-731

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Abstract

Von Hippel-Lindau disease (VHL disease) is an inherited neoplasia syndrome. VHL disease which frequently complicates pancreatic lesions is rarely diagnosed by existence of pancreatic involvements. We report two cases of VHL disease with pancreatic lesions. The first patient was a 40-year-old woman. Adrenal pheochromocytoma, spinal hemangioblastoma and pancreatic endocrine tumor were resected. The second case was a 68-year-old woman with past surgical histories included cerebellar and spinal hemangioblastoma. Subtotal pancreatectomy was performed for multiple serous cystadenoma. IPMN which has been never reported as pancreatic involvement of VHL disease were documented by imaging diagnosis in the first case, and by histological examination in the second case. We considered VHL disease from coexistent multiple tumors include pancreatic involvements and finally diagnosed by genetic examination in both cases. Care should be taken regarding the patient's right for treatment against for the genetic disease. We hold a genetic conference composed of multidisciplinary team. Consequently we detected another VHL disease patient from patient's family.

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© 2008 by The Japanese Society of Gastroenterology
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