A case of multiple pancreatic neuroendocrine tumors with von Hippel-Lindau disease

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  • MINAMIDE Tatsunori
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • WADA Masaya
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • TANIGUCHI Yohei
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • FUKUSHIMA Masashi
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • MORITA Shuko
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • SHIMENO Naoto
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • INOUE Satoko
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • TEI Hiroshi
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • SUGINOSHITA Yoshiki
    Department of Gastroenterology, Kobe City Medical Center General Hospital
  • IMAI Yukihiro
    Department of Pathology, Kobe City Medical Center General Hospital
  • INOKUMA Tetsuro
    Department of Gastroenterology, Kobe City Medical Center General Hospital

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Other Title
  • von Hippel-Lindau病に合併した多発性膵神経内分泌腫瘍の1例

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Description

A 19-year-old woman, who presented with excessive sweating and hypertension, was diagnosed with multiple pancreatic neuroendocrine tumors (PNET) and adrenal pheochromocytomas. Based on tumors in multiple organs and her family history, we diagnosed von Hippel-Lindau disease. Distal pancreatectomy (body-tail), enucleation of the tumor in the pancreatic head, and adrenalectomy were performed, avoiding total pancreatectomy in terms of preservation of pancreatic function. Histopathological examination revealed multiple PNETs in addition to those recognized before the surgery, but they were thought to be too small to detect. VHL patients often have multiple tumors including PNET synchronously and metachronously, therefore careful consideration of the operative course and follow-up is required.

Journal

  • Suizo

    Suizo 31 (2), 150-157, 2016

    Japan Pancreas Society

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