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  • Surgical Pathology Criteria

    Pancreatic Lesions of von Hippel Lindau Syndrome


    • Serous cystic lesions and endocrine lesions may be seen in the pancreas of patients with von Hippel Lindau syndrome

    Diagnostic Criteria

    • A range of serous cystic lesions may be seen in 35-80% of patients
    • Well differentiated neuroendocrine (islet cell) tumors seen in 10-17%
      • Over half have local invasion or metastasis
      • Over half have clear cell features
      • May be multiple
      • Endocrine microadenomas reported in 72% of pancreata resected for a macroscopic endocrine tumor (Perigny)
        • 5 mm or less in diameter
        • Frequently multiple
        • Not clear if these are also present in pancreata without macroscopic tumors
    • No mucinous lesions reported


    • Treatment may not be required for cystic lesions
      • No progression reported
    • Endocrine neoplasms may metastasize to local lymph nodes
      • Not clear if distant metastases occur
    • Cystic transformation of the pancreas has also been reported in a variety of other congenital syndromes

    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting : March 23, 2007
    Updates: January 12, 2008; April 26, 2009; May 25, 2009; June 20, 2009


    Cystic Pancreatic Lesions


    • Neumann HP, Dinkel E, Brambs H, Wimmer B, Friedburg H, Volk B, Sigmund G,
      Riegler P, Haag K, Schollmeyer P, et al. Pancreatic lesions in the von Hippel-Lindau syndrome. Gastroenterology. 1991 Aug;101(2):465-71.
    • Basturk O, Coban I, Adsay NV. Pancreatic cysts: pathologic classification, differential diagnosis, and clinical implications. Arch Pathol Lab Med. 2009 Mar;133(3):423-38.
    • Périgny M, Hammel P, Corcos O, Larochelle O, Giraud S, Richard S, Sauvanet A, Belghiti J, Ruszniewski P, Bedossa P, Couvelard A. Pancreatic endocrine microadenomatosis in patients with von Hippel-Lindau disease: characterization by VHL/HIF pathway proteins expression. Am J Surg Pathol. 2009 May;33(5):739-48.
    • Corcos O, Couvelard A, Giraud S, Vullierme MP, Dermot O'Toole, Rebours V, Stievenart JL, Penfornis A, Niccoli-Sire P, Baudin E, Sauvanet A, Levy P, Ruszniewski P, Richard S, Hammel P. Endocrine pancreatic tumors in von Hippel-Lindau disease: clinical, histological, and genetic features. Pancreas. 2008 Jul;37(1):85-93.

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