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Von Hippel-Lindau disease presenting as pancreatic neuroendocrine tumour

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Abstract

A 21-year-old woman with a family history of von Hippel-Lindau disease presented with a mass in the head of the pancreas. Light microscopic features of the tumour suggested neuroendocrine differentiation and although it displayed positive immunostaining for the antigens expected in a neuroendocrine neoplasm, S-100 staining was also present. This unusual feature prompted further evaluation by routine and post-embedding protein-A gold immunoelectron microscopy, which demonstrated the presence of neuroendocrine granules. Tumour cell DNA content was normal by flow cytometry. Although this patient exhibited no other signs of von Hippel-Lindau disease, the presence of a pancreatic tumour with neuroendocrine differentiation demonstrated that she was affected. Future surveillance and genetic counselling will be influenced by this diagnosis.

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Authors and Affiliations

  1. Department of Pathology, University of Vermont College of Medicine, VT 05405-0068, Burlington, Vermont, USA

    S. Mount, D. Weaver & D. Taatjes

  2. Department of Pediatrics, University of Vermont College of Medicine, Burlington, Vermont, USA

    W. McKinnon

  3. Department of Surgery, University of Vermont College of Medicine, Burlington, Vermont, USA

    J. Hebert

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  1. S. Mount
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  2. D. Weaver
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  3. D. Taatjes
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  4. W. McKinnon
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  5. J. Hebert
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Mount, S., Weaver, D., Taatjes, D. et al. Von Hippel-Lindau disease presenting as pancreatic neuroendocrine tumour. Vichows Archiv A Pathol Anat 426, 523–528 (1995). https://doi.org/10.1007/BF00193177

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  • DOI: https://doi.org/10.1007/BF00193177

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