Neuropathological Characteristics of Progression after Prolonged Response to Bevacizumab in Multifocal Hemangioblastoma

Abstract

Background: Antiangiogenic treatment has been explored in few patients with hemangioblastoma after failure of surgery and radiotherapy. Case Report: We present the first histopathological follow-up study of a bevacizumab-responsive hemangioblastoma that eventually progressed. For a period of 12 months, therapy with bevacizumab achieved a clinical response and radiological stabilization in a patient with progressive multifocal central nervous system (CNS) hemangioblastoma. Subsequently, selected tumor sites showed radiological progression, in particular, the formation of an intramedullary lesion of the initially predominantly leptomeningeal disease. Histology showed diffuse dural invasion by the hemangioblastoma accompanied with a relatively reduced cell density compared to the preserved vessel structures. Conclusion: The pattern of progression upon vascular endothelial growth factor (VEGF)-targeting antiangiogenic treatment in hemangioblastoma may involve increased tumor invasiveness.

Cervicomedullary hemangioblastoma treated with bevacizumab

“… 6–8 However, in certain instances, clinical and radiographical progression was observed. 7 , 8 Our case highlights the efficacy of limited bevacizumab therapy for intracranial hemangioblastomas unsuccessfully treated with surgery and radiosurgery.…”

Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review

“…After an extensive review of the literature in PubMed and Google Scholar using the terms “Hemangioblastoma,” “Hemangioblastomatosis,” “Leptomeningeal dissemination,” and “von Hippel–Lindau,” we found 30 similar cases reported in the past 46 years [ Table 1 ]. [ 1 - 4 , 6 , 8 , 10 , 13 , 15 , 17 , 19 , 20 , 22 - 27 , 30 , 32 , 33 ]…”

“…One patient was treated with Interferon-2 α and minocycline,[ 33 ] sunitinib was used in one patient,[ 27 ] and three patients were treated with bevacizumab. [ 8 , 24 , 30 ]…”

Metastasis of Renal Cell Carcinoma to Spinal Hemangioblastoma in a Patient with von Hippel–Lindau Disease: A Case Report

“… 4) Hemangioblastomas highly express vascular endothelial growth factor (VEGF). 14) Anti-VEGF agents, such as bevacizumab 15 , 16) and pazopanib, 17 , 18) have the potential to inhibit tumor growth of unresectable hemangioblastoma in some cases. The inactivation of the VHL tumor-suppressor gene is a specific genetic change in clear-cell RCC.…”