CNS tumor
Other tumors

Topic Completed: 1 January 2006

Minor changes: 23 February 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Hemangioblastoma [title] CNS

Nat Pernick, M.D.
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Cite this page: Pernick N. Hemangioblastoma. website. Accessed March 8th, 2021.
Definition / general
  • WHO grade I of IV (benign); slow growing and indolent but symptoms due to mass effect and peritumoral edema
  • 1 - 2% of intracranial tumors
  • Often in cerebellum; also spinal cord, meninges
  • Either part of von Hippel-Lindau disease (25 - 30%, inherited mutation of VHL gene on 3p25-26; autosomal dominant, hemangioblastomas of cerebellum and retina, cysts of liver and pancreas, pheochromocytoma, kidney tumors) or sporadic (often with somatic mutation of VHL gene)
  • Loss of VHL promotes increased production of vascular endothelial growth factor and erythropoietin
  • May be associated with loss of unknown tumor suppressor gene at 22q13 (Hum Pathol 2004;35:1105)
  • VHL patients often have new multiple, small, remote tumors (Brain Tumor Pathol 2004;21:75)
  • Note: obtain frozen section from mural nodule, NOT cyst wall
  • References: eMedicine: von Hippel-Lindau Disease {Accessed 1 August 2018]
Case reports
Gross description
  • Well circumscribed mural nodule (containing tumor) associated with large fluid filled cyst
Gross images

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Cerebellar tumor

Microscopic (histologic) description
  • Proliferation of capillaries with variable sized, closely packed, thin walled vessels and large neoplastic stromal cells with pink to clear foamy cytoplasm with fine vacuoles containing PAS+ lipid
  • Nuclei are hyperchromatic; cyst wall has gliosis and Rosenthal fibers (resembling pilocytic astrocytoma)
  • Numerous mast cells in tumor mass (Folia Neuropathol 1999;37:138), usually no atypia
  • No fibrillar cells, no necrosis and no / rare mitotic figures
Cytology description
  • Nonfibrillar stromal cells with large nuclei and vacuoles
Positive stains
Negative stains
Electron microscopy description
  • Variable secretory granules
Molecular / cytogenetics images

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Idiogram of VHL mutation

Differential diagnosis
  • Endocrine neoplasm (metastatic): clinical history; has endocrine cells in addition to prominent vascularity
  • Fibrillary astrocytoma: at frozen section due to bursting from lipid
  • Renal cell carcinoma: nuclear atypia, large nucleoli, mitotic figures, cytokeratin+, EMA+, NSE- and inhibin-
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