Multiple endocrine neoplasms and serous cysts of the pancreas in a patient with von Hippel-Lindau disease.
BACKGROUND
Patients with the von Hippel-Lindau (VHL) syndrome have an increased risk of developing pancreatic endocrine neoplasms.


METHODS
A 40-year-old female with a past history of bilateral adrenal pheochromocytomas, whose brother had a history of a malignant pheochromocytoma, was referred to our institution with symptoms of adrenal crisis including general fatigue and insomnia in spite of increasing predonisolone therapy. Screening by ultrasonogram and computed tomography demonstrated multiple well-demarcated, enhancing nodules (maximum, 3 cm) in the head and tail of pancreas and multiple cystic lesions throughout the entire pancreas. Although this patient met the criteria for VHL, magnetic resonance imaging for her brain did not reveal any lesions. Pancreatoduodenectomy with partial distal pancreatectomy was performed. Histologic examination of the resected pancreas revealed multiple well-differentiated endocrine neoplasms accompanied with multiple serous cysts. The patient remains free of disease 5 years after surgery.
Matsubayashi
Hiroyuki
H
Division of Endoscopy, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan. h.matsubayashi@scchr.jp
Uesaka
Katsuhiko
K
Kanemoto
Hideyuki
H
Sugiura
Teiichi
T
Mizuno
Takashi
T
Sasaki
Keiko
K
Ono
Hiroyuki
H
Hruban
Ralph
R
D000310:Adrenal Gland Neoplasms / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D005260:Female; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000473:pathology*; D010181:Pancreatic Cyst / Q000150:complications / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology*; D010673:Pheochromocytoma / Q000601:surgery; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000473:pathology*