title: [Case of von Hippel-Lindau disease diagnosed by detection of multiple pancreatic endocrine tumors and renal tumor 13 years after bilateral adrenalectomy].
abstract: von Hippel-Lindau (VHL) syndrome is an inherited neoplastic syndrome caused by abnormity of the VHL gene found on the short arm of the chromosome 3. We reported a case of VHL disease diagnosed by the detection of multiple pancreatic endocrine tumors and renal tumor 13 years after bilateral adrenalectomy. A 40-year-old man presented with multiple pancreas tumors (maximum size 42 mm in diameter) detected by screening abdominal ultrasonography. A 23 mm renal tumor was detected by contrast computed tomography scan at that time. His past history included left retinal angioma (age 15) and bilateral adrenal pheochromocytoma (age 27). VHL was diagnosed by genetic testing. Endoscopic ultrasound-guided fine-needle aspiration biopsy of the pancreas tumor was performed, and tumor was diagnosed as an endocrine tumor. After diagnosis, distal pancreatectomy (body-tail) was performed. This was a didactic case indicating that we should suspect VHL syndrome based on past history and family history and follow such cases up strictly.
authors.affiliation: Department of Gastroenterology, Kameda Medical Center, Japan. nakajisou1978@yahoo.co.jp
authors.forename: So
authors.initials: S
authors.lastname: Nakaji
authors.forename: Nobuto
authors.initials: N
authors.lastname: Hirata
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Junko
authors.initials: J
authors.lastname: Saito
authors.forename: Toshiyasu
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authors.lastname: Shiratori
authors.forename: Akiko
authors.initials: A
authors.lastname: Tomonari
authors.forename: Masami
authors.initials: M
authors.lastname: Inase
authors.forename: Shinako
authors.initials: S
authors.lastname: Tochitani
authors.forename: Maiko
authors.initials: M
authors.lastname: Iwata
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Fukatsu
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Fujii
authors.forename: Junro
authors.initials: J
authors.lastname: Kataoka
authors.forename: Naoko
authors.initials: N
authors.lastname: Tamaki
authors.forename: Jun
authors.initials: J
authors.lastname: Ohmori
authors.forename: Eiji
authors.initials: E
authors.lastname: Ishii
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ito
authors.forename: Satoshi
authors.initials: S
authors.lastname: Wakasugi
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D002893:Chromosomes, Human, Pair 3 / Q000235:genetics; D006391:Hemangioma; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis*; D008297:Male; D009378:Neoplasms, Multiple Primary; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000601:surgery; D010673:Pheochromocytoma / Q000601:surgery; D019572:Retinal Neoplasms; D013997:Time Factors; D006623:von Hippel-Lindau Disease / Q000175:diagnosis* / Q000235:genetics