Sporadic hemangioblastoma of the CNS and von Hippel-Lindau diseases-updated results

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SMRČKA Martin NOVOTNÝ Ondřej SMRČKA Vladimír GAILLYOVÁ Renata KŘEPELOVÁ Anna

Rok publikování 2002
Druh Článek ve sborníku
Konference Cancer Detection and Prevention
Fakulta / Pracoviště MU

Lékařská fakulta

Citace
Obor Neurologie, neurochirurgie, neurovědy
Klíčová slova hemangioblastoma; Hippel-Lindau; VHL
Popis To distinguish Von Hippel-Lindau (VHL) disease from a sporadic case of hemangioblastoma seems to be crucial for the management and follow up of these patients. A new diagnostic protocol on the basis of gene analysis have been started in oru institution. Disclosing a VHL case among those with only a solitary hemangioblastoma in the time of presentation requires an active detection for the mutated VHL gene. The occurrence of a renal cell carcinoma is very likely in VHL patients and the mortality of it has exceeded that of hamangioblastoma due to a better surgical results. Close life-long observation is needed to recognize the carcinoma soon and also for some potential for recurrent or de novo growth of hemangioblastoma.
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