History of Management of Infratentorial Hemangioblastomas in Children

Understanding of Disease

  • 1895 – Von Hippel: In 1895 von Hippel published the description of angiomatosis of the retina of the eye (60).
  • 1926 – Lindau: In 1926 Lindau, a Swedish pathologist, described a familial condition including tumors of the blood vessels of the eye and of the CNS (61).  Cushing subsequently coined the term Lindau’s disease to describe a hemangioma of the cerebellum that he operated on in a patient who also had a hemangioma of the retina (62).

Technological Development

  • Better classification using immunochemistry: Initially classified among “tumors of blood vessel origin” because of their characteristic capillary network, hemangioblastomas currently are classified among “tumors of the meninges” as a result of the development of immunohistochemistry.
  • Genetic markers for VHL: Molecular pathology analysis has determined the association with VHL.  
  • Neuroradiology for better diagnosis and treatment: Progress in neuroimaging has made the diagnosis of hemangioblastoma very accurate. Interventional radiology may aid surgical management through preoperative embolization.  

Surgical Technique