Hemangioblastomas are histologically benign
tumors, occurring almost exclusively in the Posterior
Cranial Fossa. These tumors may occur
sporadically although 20% occur as part of von
Hippel-Lindau disease. They account for 7% of primary
Posterior Cranial Fossa tumors in adults and less than 2.5% of all
primary Central Nervous System neoplasms.
Von Hippel-Lindau Disease is an inherited multi-system
disorder characterized by masses of abnormal growth of blood vessels.
A reliable source for additional information regarding von
Hippel-Lindau Disease is available at the
National Institutes of Nervous Diseases website.
Hemangioblastomas are typically found in adults
and are very rare in children with the majority becoming
symptomatic during the third to fifth decades. They are
vascular tumors of unknown origin, except in patients with von
A majority of Hemangioblastomas occur in the Posterior Cranial
Fossa. Common symptoms with any "mass lesion" in this location
The mass which constitutes Hemangioblastoma is found on either
CT or MRI scan. These scans often recognize the
evidence of acute or previous hemorrhage either into or around
the tumor. CT angiogram or MR Angiograms can be useful
in demonstrating the intense vascularity of these
- Dysequilibrium (Difficulty with balance)
In spite of the fact that Hemangioblastomas are the cause of
death in 82% of patients with von Hippel-Lindau syndrome,
these tumors are potentially surgically curable (although as many as
25% recur following surgical resection.) It is
exceptionally rare for a patient to succumb from disseminated
Appropriately aggressive treatment can be curative and
requires the removal of the mural tumor nodule (the
nodule on the wall of the cyst from which the cystic fluid is
formed), lest the cysts recur. Removal of the cyst wall is not
necessary to effect a "cure".
Radiation therapy is not useful for these tumors and does not
prevent recurrence after subtotal removal.
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This page last edited on 2/19