8.1 - Tumors of the Retina and Optic Disc

Abstract

Retinal and optic disc tumors comprise a group of benign and malignant conditions. Retinal tumors include neural tumors (retinoblastoma), glial tumors (retinal astrocytic hamartoma, acquired retinal astrocytoma, and solitary circumscribed retinal astrocytic proliferation), vascular tumors (retinal hemangioblastoma, cavernous hemangioma, racemose hemangioma, and vasoproliferative tumor), vitreoretinal lymphoma, and retinal metastasis. Optic disc tumors comprise optic disc melanocytoma, hemangioblastoma, and metastasis. Retinal and optic disc tumors are usually benign, except for retinoblastoma, vitreoretinal lymphoma, and retinal and optic disc metastases. Some of these tumors, including retinal astrocytic hamartoma, hemangioblastoma, racemose hemangioma, and possibly cavernous hemangioma, can be associated with oculoneurocutaneous syndromes (phakomatoses). Diagnostic methods used include indirect ophthalmoscopy, ultrasonography, optical coherence tomography, optical coherence tomography angiography, fluorescein angiography, fundus autofluorescence imaging, fine-needle aspiration biopsy, and pars plana vitrectomy. Treatment methods are diverse and comprise observation, laser photocoagulation, transpupillary thermotherapy, cryotherapy, photodynamic therapy, plaque radiotherapy, external beam radiotherapy to the eye/orbit, intravitreal anti–vascular endothelial growth factor and chemotherapy injections, intraarterial chemotherapy, enucleation, exenteration, and systemic chemotherapy and radiotherapy.