Retinal Capillary Hemangioblastoma: A Comprehensive Review on Treatments

Retinal capillary hemangioblastoma (RCH) is a benign tumor that frequently appears as the first manifestation in patients with von Hippel-Lindau (VHL) disease, potentially resulting in significant vision loss. Thus, recognizing and managing it promptly is crucial.

New imaging techniques, including widefield optical coherence tomography (OCT) and OCT angiography, improve diagnostic accuracy and monitoring, facilitating early treatment and better prediction of visual outcomes. While traditional therapies such as laser photocoagulation, cryotherapy, and vascular endothelial growth factor inhibitors serve as the cornerstones of RCH therapy, new approaches, including tyrosine kinase inhibitors and hypoxia-inducible factor inhibitors, also exhibit promising results in treating resistant or recurrent tumors. Furthermore, genetic testing and counseling are beneficial for identifying patients linked to VHL disease, allowing early detection of systemic manifestations of this syndrome and enabling proper therapeutic management.

This review consolidates the epidemiology, pathophysiology, clinical imaging, diagnostic evaluation, and treatment of RCH, emphasizing new insights pertinent to clinical practice and patient care.