9161/1 Angioblastoma


Related terms

Haemangioblastoma

Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

Hemangioblastomas are slowly growing, highly vascular tumour of adults, occurring in the cerebellum, (63%), spinal cord (32%), and brain stem (5%) 1
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



2
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Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D (2001)
Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease.
Neurosurgery 48: 55-62; discussion 62-3



. Histologicylly they are consist of neoplastic stromal cells and small blood vessels. While the majority of haemangioblastomas are sporadic, 10-20% occur in the setting of of the dominantly inherited von Hippel-Lindau syndrome 3
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Wanebo JE, Lonser RR, Glenn GM, Oldfield EH (2003)
The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease.
J Neurosurg 98: 82-94



4
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Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P (1989)
Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome.
J Neurosurg 70: 24-30



. Most hemangioblastomas present as cystic lesions with a solid vascular nodule that abuts a pial surface, but a smaller number of them (40%) are completely solid 5
Click to access Pubmed
Wanebo JE, Lonser RR, Glenn GM, Oldfield EH (2003)
The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease.
J Neurosurg 98: 82-94



6
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de la Monte SM, Horowitz SA (1989)
Hemangioblastomas: clinical and histopathological factors correlated with recurrence.
Neurosurgery 25: 695-8



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Despite being benign in nature, hemangioblastomas can cause significant neurological deficits and are associated with a high morbidity and mortality if not treated in time
7
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Asthagiri AR, Mehta GU, Zach L, Li X, Butman JA, Camphausen KA, Lonser RR (2010)
Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease.
Neuro Oncol 12: 80-6



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