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Scientific article
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Recurrent multiple CNS hemangioblastomas with VHL disease treated with pazopanib: a case report and literature review

Published inCNS oncology, vol. 4, no. 6, p. 387-392
Publication date2015
Abstract

Hemangioblastoma is a rare benign neoplasm, accounting for less than 2% of all primitive brain tumors. It may arise sporadically in a solitary form, or associated with Von Hippel-Lindau (VHL) disease with multiple tumors. Surgery is the mainstay treatment, but management is challenging in case of recurrent and/or multiple tumors. VHL protein is defective in both forms of hemangioblastoma, leading to the accumulation of hypoxia-inducible factor, stimulating angiogenesis via VEGF and PDGF mainly. Here, we report a 37-year-old woman's case with recurrent and rapidly progressive VHL-associated hemangioblastomas, causing severe disability. She was treated 24 months with pazopanib, a multityrosine kinase inhibitor (TKI) targeting VEGF and PDGF-β pathways. Despite moderate radiological changes, progressive improvement in her clinical condition persisting over 3 years was observed. Inhibiting angiogenesis is a therapeutic option that may improve the quality of life and the autonomy of VHL patients disabled with multiple hemangioblastomas.

Citation (ISO format)
MIGLIORINI, Denis et al. Recurrent multiple CNS hemangioblastomas with VHL disease treated with pazopanib: a case report and literature review. In: CNS oncology, 2015, vol. 4, n° 6, p. 387–392. doi: 10.2217/cns.15.22
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ISSN of the journal2045-0907
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