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

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Pages (from-to)197-202
Number of pages6
JournalJournal of Gastrointestinal Cancer
Issue number3
StatePublished - Sep 2010


  • Endocrine neoplasm
  • Pancreatic
  • Pheochromocytoma
  • Serous cyst
  • Von hippel-lindau syndrome

ASJC Scopus subject areas

  • Oncology
  • Gastroenterology


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