What causes a hemangioblastoma?
Although the exact cause of hemangioblastoma is unknown, its presence in various clinical syndromes may suggest an underlying genetic abnormality. The genetic hallmark of hemangioblastomas is the loss of function of the VHL gene.
What is hemangioblastoma?
Hemangioblastomas are benign tumors that arise from the linings of blood vessels. Hemangioblastomas can form in the brain, spinal cord, and retina (back of the eye).
How is hemangioblastoma treated?
Symptomatic cerebellar or brainstem hemangioblastomas are typically treated by surgical removal through a sub-occipital or Retromastoid Craniotomy. The goal of hemangioblastoma surgery is to remove the enhancing tumor nodule completely which is able to be accomplished in 80-90% of patients.
Is hemangioblastoma curable?
If a hemangioblastoma can be completely removed and is not associated with von Hippel Lindau disease, the patient can be cured. If the entire tumor cannot be resected, it is likely that the tumor will re-grow or that additional cysts will form. However, most hemangioblastomas can be safely removed.
Do Hemangioblastomas grow back?
If the entire tumor is removed, your condition is cured. However, if part of the tumor remains, it can regrow and you may continue to experience symptoms.
How fast do Hemangioblastomas grow?
Hemangioblastomas grew in a stuttering pattern. (mean growth period 13 +/- 15 months, mean quiescent period 25 +/- 19 months).
Are Hemangioblastomas benign?
Hemangioblastomas are vascular tumors of unknown origin. Occurring almost exclusively in the posterior fossa, these benign tumors most often occur sporadically, but 20 percent occur as part of von Hippel-Lindau disease.
What is the clinical presentation of retinal capillary hemangioblastoma?
The clinical presentation of retinal capillary hemangioblastoma varies greatly depending on the size and location of the tumor. In a large cross sectional study performed by the National Eye Institute where 335 VHL patients were enrolled, RCH was was unilateral in 42% of cases and bilateral in 58%.
What kind of tumor is a hemangioblastoma?
Pathology. Hemangioblastoma is actually a capillary hemangioma and, despite the name with the affix of “blastoma”, it is a low grade (WHO grade I) lesion (note that the calvarial hemangioma is a cavernous hemangioma). The tumor is usually well circumscribed with a highly vascular mural nodule almost always abutting pial layer…
How are hemangioblastomas associated with von Hippel-Lindau syndrome?
Bilateral or multiple retinal hemangioblastomas are associated with von Hippel-Lindau (VHL) syndrome, and patients should be evaluated for this condition using brain and renal imaging as well as genetic testing (Table 1). Solitary retinal hemangioblastomas may also be associated with VHL syndrome.
Can a retinal hemangioblastoma cause profound visual loss?
When retinal hemangioblastoma is located at the optic disc, the mass can masquerade as papillitis, and the feeder vessels are often not visible. Regardless of tumor location, accumulation of subretinal fluid with exudation can lead to profound visual loss.