Case of von Hippel-Lindau disease diagnosed by detection of multiple pancreatic endocrine tumors and renal tumor 13 years after bilateral adrenalectomy

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.

Medienart:

Artikel

Erscheinungsjahr:

2010

Erschienen:

2010

Enthalten in:

Zur Gesamtaufnahme - volume:107

Enthalten in:

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology - 107(2010), 12 vom: 01. Dez., Seite 1978-87

Sprache:

Japanisch

Beteiligte Personen:

Nakaji, So [VerfasserIn]
Hirata, Nobuto [VerfasserIn]
Kobayashi, Masayoshi [VerfasserIn]
Saito, Junko [VerfasserIn]
Shiratori, Toshiyasu [VerfasserIn]
Tomonari, Akiko [VerfasserIn]
Inase, Masami [VerfasserIn]
Tochitani, Shinako [VerfasserIn]
Iwata, Maiko [VerfasserIn]
Fukatsu, Kazuhiro [VerfasserIn]
Fujii, Hiroyuki [VerfasserIn]
Kataoka, Junro [VerfasserIn]
Tamaki, Naoko [VerfasserIn]
Ohmori, Jun [VerfasserIn]
Ishii, Eiji [VerfasserIn]
Ito, Hiroshi [VerfasserIn]
Wakasugi, Satoshi [VerfasserIn]

Themen:

Case Reports
English Abstract
Journal Article

Anmerkungen:

Date Completed 03.03.2011

Date Revised 28.09.2020

published: Print

Citation Status MEDLINE

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM204179688