• Ann Pathol · Mar 2000

    Case Reports

    [Pancreatic neuroendocrine tumors and von Hippel-Lindau disease].

    • C Musso, F Paraf, B Petit, F Archambeaud-Mouveroux, D Valleix, and F Labrousse.
    • Service d'Anatomie Pathologique, Centre Hospitalier Régional Universitaire Dupuytren, 87042 Limoges, France.
    • Ann Pathol. 2000 Mar 1; 20 (2): 130-3.

    AbstractPancreatic neuroendocrine tumors are rare in von Hippel-Lindau disease, most often asymptomatic, nonfunctioning, non secreting, and benign. We report a case of low grade malignant pancreatic, secreting and asymptomatic neuroendocrine tumors, occurring in a 27 year old woman in the setting von Hippel-Lindau disease with recurrent pheochromocytoma, retinal and medullary hemangioblastomas, paraganglioma of the carotid body and ovarian cystadenoma. Neuroendocrine pancreatic tumors of von Hippel-Lindau disease are often constituted by clear cells, in the contrary of other neuroendocrine tumors of the pancreas. Occurrence of a pancreatic neuroendocrine tumor, especially in association with pheochromocytoma, may be misdiagnosed with a type 2 multiple endocrine neoplasia syndrom instead of von Hippel-Lindau disease.

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