The major differential diagnosis of leukocoria is Toxocara endophthalmitis , Coats' disease, retinal dysplasia, persistent hyperplastic primary vitreous, and retinal detachment of unknown cause. The presence of intraocular calcium is an important clinical findings because few simulating lesions in the differential diagnosis become mineralized. Retinocytoma, the benign variant of retinoblastoma, has the appearance of a treated retinoblastoma. Gliomas of the retina are rare and usually are associated with tuberous sclerosis or neurofibromatosis. These whitish tumors often contain calcium and may resemble a small retinoblastoma.
Retinal gliomas are probably hamartomatous malformations and have limited growth potential.
Retinal hemangiomas are found in von Hippel-Lindau syndrome and appear as a pink retinal mass. These vascular tumors typically have a large retinal feeder vessel and vein. Intraocular Lymphoma Primary intraocular lymphoma may mimic inflammatory conditions of the eye, but some cases also have a highly characteristic clinical appearance. Some primary intraocular lymphomas appear to originate in the space between retinal pigment epithelium and Bruch's membrane. Limited vitreous reaction may be present during the early stages of the disease so lesions are usually visible.