Mechanisms, indications and results of salvage systemic therapy for sporadic and von Hippel-Lindau related hemangioblastomas of the central nervous system

Jody Filippo Capitanio, Elena Mazza, Micaela Motta, Pietro Mortini, Michele Reni

Research output: Contribution to journalArticle

Abstract

Hemangioblastomas (HBs) are rare indolent vascular tumors that may occur sporadically or in association with von Hippel-Lindau (VHL) disease. Total neurosurgical resection is the standard upfront approach providing long-term tumor control. At time of tumor recurrence, second surgery, radiosurgery or radiotherapy are the main therapeutic strategies.Limited information is available on the role of pharmacological strategies. Anti-angiogenic agents, particularly multitarget tyrosine kinase inhibitors (semaxanib, sunitinib, vatalanib), thalidomide and interferon alfa-2a are currently the most widely studied strategies to prolonge disease stability.Salvage therapy with anti-angiogenetic drugs may be of benefit in some patients who are not suitable for surgery, radiosurgery or radiotherapy, with progressive or recurrent hemangioblastoma especially those located in retina, since anti-angiogenetic therapy may delay tumor progression. This strategy warrants prospective evaluation in a clinical trial.

Original languageEnglish
Pages (from-to)69-84
Number of pages16
JournalCritical Reviews in Oncology/Hematology
Volume86
Issue number1
DOIs
Publication statusPublished - Apr 2013

Keywords

  • Anti-angiogenetic drugs
  • Guidelines
  • HB follow-up
  • Interferon-alpha-2a
  • Pharmacologic treatment for HBs
  • Salvage systemic therapy
  • Semaxanib
  • Sporadic HB
  • Su5416
  • Thalidomide
  • Therapeutic options
  • Tyrosine kinase inhibitors
  • Vatalanib
  • VHL related HB

ASJC Scopus subject areas

  • Oncology
  • Hematology
  • Geriatrics and Gerontology

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