title: Serous cystic neoplasms of the whole pancreas in a patient with von Hippel-Lindau disease.
abstract: We describe here a case of von Hippel-Lindau (VHL) disease with a serous cystic neoplasm of the whole pancreas. The patient was a 35-year-old woman suffering from a palpable abdominal tumor. She had a history of hemangioblastomas of the cerebellum. CT revealed large solid tumors in the pancreatic head and body, and multiple cystic lesions in the whole pancreas as well as a right renal tumor. When endoscopic retrograde cholangiopancreatography (ERCP) was performed, bleeding from the duodenal papilla was detected. Since she had some distinguishing clinical features, the diagnosis of VHL disease was made. The preoperative diagnosis of the pancreatic lesion was serous cystic neoplasms with hemosuccus pancreaticus and total pancreatectomy was performed. Histological examination of the specimen revealed serous cystic neoplasms which occupied the entire pancreas. VHL cases operated on for serous cystic neoplasms of the entire pancreas are very rare.
authors.affiliation: Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan. atsushih@med.tohoku.ac.jp
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kanno
authors.forename: Kennichi
authors.initials: K
authors.lastname: Satoh
authors.forename: Shin
authors.initials: S
authors.lastname: Hamada
authors.forename: Morihisa
authors.initials: M
authors.lastname: Hirota
authors.forename: Atsushi
authors.initials: A
authors.lastname: Masamune
authors.forename: Fuyuhiko
authors.initials: F
authors.lastname: Motoi
authors.forename: Shinichi
authors.initials: S
authors.lastname: Egawa
authors.forename: Michiaki
authors.initials: M
authors.lastname: Unno
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Ishida
authors.forename: Kenji
authors.initials: K
authors.lastname: Kimura
authors.forename: Taro
authors.initials: T
authors.lastname: Shuin
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa
mesh_terms: D000328:Adult; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D002760:Cholangiopancreatography, Endoscopic Retrograde; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D008279:Magnetic Resonance Imaging; D018297:Neoplasms, Cystic, Mucinous, and Serous / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis / Q000601:surgery