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Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease

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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.

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Acknowledgment

The authors thank Dr. Masaya Onose, Dr. Eisho Yoshikawa, and Miss. Yukie Kurihara for their thoughtful care of the patient.

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Authors and Affiliations

  1. Division of Endoscopy, Shizuoka Cancer Center, 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka, 411-8777, Japan

    Hiroyuki Matsubayashi & Hiroyuki Ono

  2. Division of Hepato-biliary-pancreatic Surgery, Shizuoka Cancer Center, Nagaizumi, Suntogun, Shizuoka, 411-8777, Japan

    Katsuhiko Uesaka, Hideyuki Kanemoto, Teiichi Sugiura & Takashi Mizuno

  3. Division of Pathology, Shizuoka Cancer Center, Nagaizumi, Suntogun, Shizuoka, 411-8777, Japan

    Keiko Sasaki

  4. The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, Baltimore, MD, 21231, USA

    Ralph Hruban

Authors
  1. Hiroyuki Matsubayashi
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  2. Katsuhiko Uesaka
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  3. Hideyuki Kanemoto
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  4. Teiichi Sugiura
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  5. Takashi Mizuno
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  6. Keiko Sasaki
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  7. Hiroyuki Ono
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  8. Ralph Hruban
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Corresponding author

Correspondence to Hiroyuki Matsubayashi.

Additional information

IRB Approval: This case report was approved by institutional review board of Shizuoka Cancer Center (code no. 21-J19-21-1-3).

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Matsubayashi, H., Uesaka, K., Kanemoto, H. et al. Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease. J Gastrointest Canc 41, 197–202 (2010). https://doi.org/10.1007/s12029-010-9134-3

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  • DOI: https://doi.org/10.1007/s12029-010-9134-3

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