Retinal Capillary Hemangioblastoma: A Comprehensive Review on Treatments

Retinal hemangioblastoma (RH) 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 wide-field optical coherence tomography (OCT) and OCT angiography (OCTA), enhance diagnostic accuracy and monitoring, aiding in early treatments and improving predictions for vision. While traditional therapies such as laser photocoagulation, cryotherapy, and VEGF inhibitors serve as the cornerstones of RH therapy, new approaches, including tyrosine kinase inhibitors (TKIs) and HIF 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 RH, emphasizing new insights pertinent to clinical practice and patient care.