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HEMANGIOBLATOMA (von HIPPEL-LINDAU DISEASE)

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 Hippel-Lindau Disease.

SYMPTOMS

A majority of Hemangioblastomas occur in the Posterior Cranial Fossa. Common symptoms with any "mass lesion" in this location include:
  • Headaches
  • Nausea/vomiting,
  • Dysequilibrium (Difficulty with balance)
  • Dizziness/vertigo

DIAGNOSIS

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 lesions.

Figure 1: MRI Scan (Gadolinium Enhanced Axial View) Left Cerebellar Hemisphere Cystic Tumor.

The "Mural Nodule" (Curved Arrow) "enhances" due to its considerable blood supply and is responsible for the formation of the fluid of the cyst. The "Nodule" must be entirely removed in order to "cure" this lesion, particularly in "Sporadically" occurring cases.

Cysts of this size cause considerable pressure on the Brain Stem which can threaten the patient's life if left untreated.


TREATMENT

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 disease.

Surgery

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

Radiation therapy is not useful for these tumors and does not prevent recurrence after subtotal removal.

ADDITIONAL INFORMATION

http://www.ninds.nih.gov/disorders/von_hippel_lindau/von_hippel_lindau.htm

http://www.ncbi.nlm.nih.gov/books/bv.fcgi?call=bv.View..ShowSection&rid=gnd.section.143



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This page last edited on 2/19

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.