Hemangioblastoma: Systemic therapy

Hemangioblastoma is a benign vascular neoplasm that arises almost exclusively in the central nervous system but is occasionally seen elsewhere. It can be associated with Hippel-Lindau disease. According to the World Health Organization classification of tumors of the nervous system, hemangioblastomas are classified as meningeal tumors of uncertain origin. Treatment approaches are usually local:  surgery,  embolization or radiosurgery.

Little is known about systemic treatment of hemangioblastoma. Chemotherapy does not appear to be beneficial and I did not find any published literature on the use of Xeloda.  Sorafenib does not appears to work. A single 2012 case report suggested that pazopanib may work but this remains to be substantiated.  Several trials of novel agents are listed on clinicaltrials.gov.

Jagannathan J, Lonser RR, Smith R, et al. Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2008; 108:210.
Kanno H, Yamamoto I, Nishikawa R, et al. Spinal cord hemangioblastomas in von Hippel-Lindau disease. Spinal Cord 2009; 47:447.
Parker F, Aghakhani N, Ducati LG, et al. Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups. J Neurooncol 2009; 93:133.
Moss JM, Choi CY, Adler JR Jr, et al. Stereotactic radiosurgical treatment of cranial and spinal hemangioblastomas. Neurosurgery 2009; 65:79.
Kano H, Niranjan A, Mongia S, et al. The role of stereotactic radiosurgery for intracranial hemangioblastomas. Neurosurgery 2008; 63:443.
N. Panakis, C. Offiah, A. Azizan, A. Roy, P. N. Plowman Sorafenib therapy and CNS hemangioblastomas in individuals with Von Hippel Lindau syndrome.  J Clin Oncol 26: 2008 (May 20 suppl; abstr 2065)
Kim BY, Jonasch E, McCutcheon IE.Pazopanib therapy for cerebellar hemangioblastomas in von Hippel-Lindau disease: case report. Target Oncol. 2012 Jun;7(2):145-9.
Yin X, Duan H, Yi Z, Li C, Lu R, Li L. Incidence, Prognostic Factors and Survival for Hemangioblastoma of the Central Nervous System: Analysis Based on the Surveillance, Epidemiology, and End Results Database. Front Oncol. 2020;10:570103. Published 2020 Sep 9. doi:10.3389/fonc.2020.570103

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