Hepatic hemangioblastoma. An unusual presentation in a patient with von Hippel-Lindau disease

Am J Surg Pathol. 1991 Jan;15(1):81-6.

Abstract

We report a case of multiple capillary hemangioblastomas of the liver occurring in a patient with von Hippel-Lindau disease and a history of previous cerebellar and spinal hemangioblastomas. Although rare examples of this tumor have previously been recorded in the pancreas, kidney, and urinary bladder, this appears to be the first recorded case with hepatic involvement. The histology and immunohistochemical appearance of this neoplasm are identical with those of the cerebellar tumor. We believe it represents a separate primary neoplasm rather than metastatic disease.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Adult
  • Diagnosis, Differential
  • Factor VIII / metabolism
  • Female
  • Glial Fibrillary Acidic Protein / metabolism
  • Hemangiosarcoma / complications*
  • Hemangiosarcoma / metabolism
  • Hemangiosarcoma / pathology
  • Humans
  • Immunohistochemistry
  • Lectins / metabolism
  • Liver / metabolism
  • Liver / pathology
  • Liver Neoplasms / complications*
  • Liver Neoplasms / metabolism
  • Liver Neoplasms / pathology
  • Phosphopyruvate Hydratase / metabolism
  • Plant Lectins*
  • Polycythemia / etiology
  • Polycythemia / pathology
  • S100 Proteins / metabolism
  • Spinal Cord Neoplasms / complications
  • Spinal Cord Neoplasms / pathology
  • von Hippel-Lindau Disease / complications*
  • von Hippel-Lindau Disease / metabolism
  • von Hippel-Lindau Disease / pathology

Substances

  • Glial Fibrillary Acidic Protein
  • Lectins
  • Plant Lectins
  • S100 Proteins
  • Ulex europaeus lectins
  • Factor VIII
  • Phosphopyruvate Hydratase