Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease
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Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease Hiroyuki Matsubayashi & Katsuhiko Uesaka & Hideyuki Kanemoto & Teiichi Sugiura & Takashi Mizuno & Keiko Sasaki & Hiroyuki Ono & Ralph Hruban
Published online: 6 February 2010 # Springer Science+Business Media, LLC 2010
Abstract Introduction Patients with the von Hippel–Lindau (VHL) syndrome have an increased risk of developing pancreatic endocrine neoplasms. Case Report 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. Keywords von Hippel–Lindau syndrome . pancreatic . endocrine neoplasm . serous cyst . pheochromocytoma
Introduction IRB Approval: This case report was approved by institutional review board of Shizuoka Cancer Center (code no. 21-J19-21-1-3). H. Matsubayashi (*) : H. Ono Division of Endoscopy, Shizuoka Cancer Center, 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan e-mail: [email protected] K. Uesaka : H. Kanemoto : T. Sugiura : T. Mizuno Division of Hepato-biliary-pancreatic Surgery, Shizuoka Cancer Center, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan K. Sasaki Division of Pathology, Shizuoka Cancer Center, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan R. Hruban The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
Occurring in one in 36,000 live births [1], patients with von Hippel–Lindau (VHL) have an increased risk of developing neoplasms of the brain, spinal cord, retina, kidney, adrenal gland, and testis. These neoplasms include hemangioblastoma, clear-cell carcinoma, pheochromocytoma, paraganglioma, endocrine neoplasms, cystic neoplasms, and seminoma. Two thirds to three quarters of patients with VHL [2–3] develop a pancreatic lesion, usually a cystic lesion (17–56%) [4, 5], rarely endocrine neoplasm (10–17%) [3, 6, 7], or combined cystic-endocrine neoplasms in 12% [3]. Pancreatic endocrine neoplasms (PENs) in patients with VHL are usually diagnosed at young age (mean, 29– 38 years), are mostly non-functioning (∼98%), often recognized as a single lesion (∼70%), and they have a significant malignant potential (∼50%) [6]. We herein report a pa
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