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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Aldape KD, Plate KH, Vortmayer AO, Zagzag D, Neumann HPH. (2007) Haemangioblastoma. von Hippel-Lindau disease and haemangioblastoma. In: Louis DH, Ohgaki H, Wiestler OD, Cawenee WK (eds) Pathology and genetics of tumors of the nervous system. WHO International Agency for Research on Cancer, Lyon, pp. 184–186/215–217
Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. (2006) Long-term natural history of hemangio-blastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg 105:248–255
Cornelius JF, Saint-Maurice JP, Bresson D, George B, Houdart E. (2007) Hemorrhage after particle embolization of hemangioblastomas: comparison of outcomes in spinal and cerebellar lesions. J Neurosurg 106:994–998
Costa LJ, Drabkin HA. (2007) Renal cell carcinoma: new developments in molecular biology and potential for targeted therapies. Oncologist 12:1404–1415
Gläsker S, Van Velthoven V. (2005) Risk of hemorrhage in hemangioblastomas of the central nervous system. Neurosurgery 57:71–76
Hes FJ, van der Luijt RB, Janssen AL, Zewald RA, de Jong GJ, Lenders JW, Links TP, Luyten GP, Sijmons RH, Eussen HJ, Halley DJ, Lips CJ, Pearson PL, van den Ouweland AM, Majoor-Krakauer DF. (2007) Frequency of Vo n Hippel-Lindau germline mutations in classic and non-classic Von Hippel-Lindau disease identifi ed by DNA sequencing, Southern blot analysis and multiplex ligation-dependent probe amplifi cation. Clin Genet 72:122–129
Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfi eld EH. (2008) Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 108:210–222
Kaelin WG Jr. (2007) The von hippel-lindau tumor suppressor protein: an update. Methods Enzymol 435:371–383
Koh ES, Nichol A, Millar BA, Ménard C, Pond G, Laperriere NJ. (2007) Role of fractionated external beam radiotherapy in hemangioblastoma of the central nervous system. Int J Radiat Oncol Biol Phys 69:1521–1526
Leung RS, Biswas SV, Duncan M, Rankin S. (2008) Imaging features of von Hippel-Lindau disease. Radiographics 28:65–79
Lonser RR, Weil RJ, Wanebo JE, DeVroom HL, Oldfi eld EH. (2003) Surgical management of spinal cord hemangioblasto-mas in patients with von Hippel-Lindau disease. J Neurosurg 98:106–116
Mittendorf EA, Evans DB, Lee JE, Perrier ND. (2007) Pheochromocytoma: advances in genetics, diagnosis, localization, and treatment. Hematol Oncol Clin North Am 21:509–525
Niemela M, Maenpaa H, Salven P, Summanen P, Poussa K, Laatikainen L, Jaaskelainen J, Joensuu H. (2001) Interferon alpha-2a therapy in 18 hemangioblastomas. Clin Cancer Res 7:510–516
Niemelä M, Lemeta S, Sainio M, et al (2000) Hemangio-blastoma of the retina: impact of von Hippel-Lindau disease. Invest Ophthalmol Vis Sci 41:1909–1915
Niemelä M, Lemeta S, Summanen P, et al (1999) Long-term prognosis of haemangioblastoma of the CNS: impact of von Hippel-Lindau disease. Acta Neurochir 141:1147–1156
Ploussard G, Droupy S, Ferlicot S, Ples R, Rocher L, Richard S, Benoit G. (2007) Local recurrence after nephron-sparing surgery in von Hippel-Lindau disease. Urology 70:435–439
Wang EM, Pan L, Wang BJ, Zhang N, Zhou LF, Dong YF, Dai JZ, Cai PW, Chen H. (2005) The long-term results of gamma knife radiosurgery for hemangioblastomas of the brain. J Neurosurg 102(Suppl):225–229
Weil RJ, Lonser RR, DeVroom HL, Wanebo JE, Oldfi eld EH. (2003) Surgical management of brainstem hemangioblasto-mas in patients with von Hippel-Lindau disease. J Neurosurg 98:95–105
Wong WT, Agrón E, Coleman HR, Tran T, Reed GF, Csaky K, Chew EY. (2008) Clinical characterization of retinal capillary hemangioblastomas in a large population of patients with von Hippel-Lindau disease. Ophthalmology 115:181–188
Woodward ER, Wall K, Forsyth J, Macdonald F, Maher ER. (2007) VHL mutation analysis in patients with isolated central nervous system haemangioblastoma. Brain 130:836–842
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-642-02874-8_16
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-02873-1
Online ISBN: 978-3-642-02874-8
eBook Packages: MedicineMedicine (R0)