Hemangioblastoma

Hemangioblastomas are most commonly composed of stromal cells in small blood vessels and usually occur in the cerebellum, brainstem or spinal cord.

[2] Hemangioblastomas can cause an abnormally high number of red blood cells in the bloodstream due to ectopic production of the hormone erythropoietin as a paraneoplastic syndrome.

In normal circumstances, pVHL is involved in the inhibition of hypoxia-inducible factor 1 α (HIF-1α) by ubiquitin mediated proteosomal degradation.

On a scan, hemangioblastoma shows as a well-defined, low attenuation region in the posterior fossa with an enhancing nodule on the wall.

[1] Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.

Contrast enhanced MRI of the conus medullaris and cauda equina of patient with VHL depicts a hemangioblastoma of the conus with extension into the nerve roots.