Retinal Vascular Tumors

Retinal vascular tumors represent at least four distinct clinical entities, which include retinal hemangioblastoma, retinal cavernous hemangiomas, retinal arteriovenous communications, Wyburn-Mason syndrome, and retinal vasoproliferative tumor. Retinal vascular tumors are (a) congenital, maintaining retinal tight junctions and hence without causing subretinal fluid or hard exudates (retinal cavernous hemangioma and retinal arteriovenous communications [Wyburn-Mason syndrome]), or (b) acquired, without retinal tight junctions and hence causing subretinal fluid or hard exudates (retinal hemangioblastoma and retinal vasoproliferative tumor). Each of these has distinctive clinical features and specific systemic associations so that accurate diagnosis is essential if patients are to receive correct prognostication and effective treatment. Retinal hemangioblastoma and retinal arteriovenous communications are described only briefly in this chapter as they are further discussed in Chap. 9 , which covers neuro-oculocutaneous syndromes (phakomatoses).