[Two cases of pancreatic tumor with von Hippel-Lindau disease].
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.
Karasaki
Hidenori
H
Division of Gastroenterologic and General Surgery, Asahikawa Medical College.
Ishizaki
Akira
A
Yanagawa
Nobuyuki
N
Nakano
Yasuhiro
Y
Sasajima
Jyunpei
J
Mizukami
Yusuke
Y
Tanno
Satoshi
S
Tokusashi
Yoshihiko
Y
Miyokawa
Naoyuki
N
Obara
Mitsuhiro
M
Goto
Junichi
J
Kino
Shuichi
S
Kono
Toru
T
Kasai
Shinichi
S
D000328:Adult; D000368:Aged; D018293:Cystadenoma, Serous / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D025202:Molecular Diagnostic Techniques; D009378:Neoplasms, Multiple Primary; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics