Abstract
Hemangioblastoma (HB) is an infrequent, benign (WHO grade I), highly vascular, well-demarcated, slowly growing, solid, or cystic neoplasm of unspecifi ed cellular origin [1]. It is confined to the central nervous system (CNS), the brain, spinal cord, and retina, rarely occurring in the nerve roots or peripheral nerves. HB accounts for about 10% of tumors of the posterior fossa, the site of its predilection, but only about 2% of all intracranial tumors. HB of the retina [1, 14, 19], originating from the inner mid-peripheral retina, is histologically identical to HB elsewhere in the CNS. Some 20% of HBs (up to 50% of retinal HBs) may be associated with Von Hippel–Lindau disease (VHL), but estimates are inaccurate because not all patients are screened for the mutations and other manifestations of VHL [1, 6, 10, 15, 20]. VHL-related HBs occur at 20–30 years of age and sporadic ones at 40–50 years.
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Jääskeläinen, J.E., Niemelä, M. (2010). Hemangioblastoma and Von Hippel–Lindau Disease. In: Tonn, JC., Westphal, M., Rutka, J.T. (eds) Oncology of CNS Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-02874-8_16
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