Use of intravitreal bevacizumab in a patient with a Von Hippel-Lindau-associated retinal haemangioblastoma of the optic nerve head: a case report

Abstract

Introduction: The optimum management of a capillary haemangioblastoma affecting the optic nerve head is not clear. A number of treatment modalities have been used to treat the tumours and their consequences. Ocular haemangioblastomas express high levels of vascular endothelial growth factor and levels have been correlated with tumour growth and activity. Treatment with vascular endothelial growth factor inhibitors would therefore seem a logical approach.

Bevacizumab for the treatment of surgically unresectable cervical cord hemangioblastoma: a case report

“…Bevacizumab is currently used for the treatment of a variety of solid tumors including recurrent glioblastomas, metastatic colorectal carcinoma, and breast cancer as well as renal cell carcinoma [ 7 ]. Intravitreal bevacizumab was attempted in previous series for the treatment of retinal hemangioblastomas with varying degrees of success [ 9 , 10 ].…”

“…In addition, a more recent report indicates that retinal hemangioblastomas may respond favorably to bevacizumab [ 9 ]. Another case report showed that treatment with intravitreal bevacizumab had no effect on a hemangioblastoma of the optic nerve head [ 10 ]. To the best of our knowledge, our report is the first to show tumor regression as well as sustained and meaningful clinical improvement in a patient with unresectable spinal cord hemangioblastoma treated with bevacizumab.…”

Neurosurgical considerations in von Hippel–Lindau disease

“…Retinal haemangioblastomas have been treated with systemic SU5416 (a VEGF receptor inhibitor) and intravitreal bevacizumab. 52,53 While macular oedema associated with the retinal haemangioblastoma improved in the former case, neither therapies were effective against the tumour. SU5416 was also used in a series of six patients with VHL by Madhusudan et al with clinical improvements in two patients (follow up period not specified).…”

Antiangiogenic Treatment for Multiple CNS Hemangioblastomas

“…Therefore, antiangiogenic treatment seems logical when complete microsurgical resection is impossible. Sunitinib is one of the most frequently used antiangiogenic agents and currently under investigation in a phase II study (ClinicalTrials.gov Identifier:NCT00589784).Sofar,noexperiencewithintravenous bevacizumab has been published; however intravitreal bevacizumabhasbeendescribedinpatientswithVHL-associatedretinalhemangioblastomas [9,10].Todate,1openphase 0 study is investigating bevacizumab in hemangioblastomas (ClinicalTrials.gov Identifier: NCT01015300). The 2 patients described here experienced an impressive and long-lasting clinicalbenefit.Theydemonstratedradiologicalstabilization of symptomatic multifocal hemangioblastomas in the brain andspinewithbevacizumab.Thefailuretoinduceobjective remissionsuggeststhatVEGFisnotasurvivalfactorforthe tumorcells,whereastheinductionofstablediseaseindicates thatVEGFmaybeagrowthfactorinthisdisease.Theclinical benefitisdifficulttoexplainbutmaybecausedbyareduction of perfusion and edema and possibly improved perfusion of adjacent brain parenchyma.…”