Hemangioblastoma

Hemangioblastoma
Hemangioblastoma
Classification and external resources

Micrograph of a cerebellar hemangioblastoma. HPS stain.
ICD-O: M9161/1
DiseasesDB 31512
eMedicine med/2991 radio/326
MeSH D018325

Hemangioblastomas (also known as capilliary hemangioblastomas[1]) are tumors of the central nervous system that originate from the vascular system usually during middle-age. Sometimes these tumors occur in other sites such as the spinal cord and retina.[2] They may be associated with other diseases such as polycythemia (increased blood cell count), pancreatic cysts and Von Hippel-Lindau syndrome (VHL syndrome). Hemangioblastomas are most commonly composed of stromal cells in small blood vessels and usually occur in the cerebellum, brain stem or spinal cord. They are classed as grade one tumors under the World Health Organisation's classification system.[1]

Contents

Diagnosis

The primary diagnosis is made with a computed tomography scan (CT scan). On a scan, hemangioblastoma shows as a well-defined, black region in the cerebellum with a white nodule on the wall. Sometimes multiple lesions are present.[2]

Treatment

The treatment for hemangioblastoma is surgical excision of the tumour. Although usually straightforward to carry out, recurrence of the tumor or more tumors at a different site develop in approximately 20% of patients.[2] Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.

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.[1]

Complications

Hemangioblastomas can cause polycythemia.

Epidemiology

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 central nervous system, they usually occur in either side of the cerebellum, the brain stem or the spinal cord.[1]

See also

Additional images

  • Micrograph of cerebellar hemangioblastoma. HPS stain.

  • Micrograph of cerebellar hemangioblastoma. HPS stain.

References

  1. ^ a b c d Louis, David N (1991). WHO Classification of Tumors of the Central Nervous System. IARC. ISBN 9283224302. 
  2. ^ a b c Lindsay, Kenneth W; Ian Bone, Robin Callander, J. van Gijn (1991). Neurology and Neurosurgery Illustrated. United States: Churchill Livingstone. ISBN 0-443-04345-0. 

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Look at other dictionaries:

  • hemangioblastoma — m. angiol. Hemangioma formado principalmente por angioblastos. Medical Dictionary. 2011. hemangioblastoma tumor …   Diccionario médico

  • hemangioblastoma — noun Any of several benign neoplasm tumours of the brain …   Wiktionary

  • hemangioblastoma — A benign neoplasm frequently arising in the cerebellum composed of capillary vessel–forming endothelial cells and stromal cells; a slowly growing tumor that affects, primarily, middle aged individuals; increased incidence in von Hippel Lindau… …   Medical dictionary

  • cerebellar hemangioblastoma — hemangioblastoma of the cerebellum, often cystic; an autosomal dominant form is associated with von Hippel Lindau disease …   Medical dictionary

  • spinal hemangioblastoma — a hemangioblastoma of the spinal cord, usually small and encapsulated and in an intramedullary location …   Medical dictionary

  • retinal hemangioblastoma — von Hippel disease …   Medical dictionary

  • cerebellar hemangioblastoma — A benign, slow growing tumor in the cerebellum (part of the brain at the back of the head), made up of abnormal blood vessel growth. People with von Hippel Landau disease have an increased risk of developing hemangioblastomas …   English dictionary of cancer terms

  • Lindau tumor — hemangioblastoma …   Medical dictionary

  • Гемангиобластома — мозжечка МКБ 1 …   Википедия

  • Obex — Brain: Obex Hind brain of a human embryo of three months viewed from behind and partly from left side …   Wikipedia

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