Hemangioblastoma

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Hemangioblastoma
cerebellar hemangioblastoma. HPS stain.
SpecialtyOncology Edit this on Wikidata

Hemangioblastomas, or haemangioblastomas, are

stromal cells in small blood vessels and usually occur in the cerebellum, brainstem or spinal cord. They are classed as grade I tumors under the World Health Organization's classification system.[2]

Presentation

Complications

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.[3]

Pathogenesis

Hemangioblastomas are composed of

transforming growth factor alpha, which act to stimulate growth of cells within the tumor.[4]

Diagnosis

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.

The primary diagnosis is made with a

computed tomography scan (CT scan). On a scan, hemangioblastoma shows as a well-defined, low attenuation region in the posterior fossa with an enhancing nodule on the wall. Sometimes multiple lesions are present.[1]

Treatment

The treatment for hemangioblastoma is

Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.[6]

Prognosis

The outcome for hemangioblastoma is very good if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed and treated early. Persons with VHL syndrome have a bleaker prognosis than those who have sporadic tumors since those with VHL syndrome usually have more than one lesion.[2]

Epidemiology

Hemangioblastoma are among the rarest central nervous system tumors, accounting for less than 2%. Hemangioblastomas usually occur in adults, yet tumors may appear in VHL syndrome at much younger ages. Men and women are approximately at the same risk. Although they can occur in any section of the

brain stem or the spinal cord.[2][7]

Additional images

  • Micrograph of cerebellar hemangioblastoma. HPS stain.
    Micrograph of cerebellar hemangioblastoma. HPS stain.
  • Micrograph of cerebellar hemangioblastoma. HPS stain.
    Micrograph of cerebellar hemangioblastoma. HPS stain.

References

External links