[Diffuse infiltrating retinoblastoma]

Abstract

We report 3 new cases of diffuse infiltrating retinoblastoma, and we review the 21 cases of the literature. Clinical features are typical but must not be confused with uveitis. The average age of onset is about 7 years, later than the usual retinoblastoma. Clinical features associate ocular redness, pseudo hypopion, iris nodules, clusters on the pupil and in the anterior chamber, opacities on the posterior face of the cornea. Hypertension appears resistant to medical treatment. The vitreous is hazy but the retina is still visible. The ophthalmoscopic examination reveals exudates covering the peripheric retina, and gray infiltrated retina. Usually there is no focal tumour mass, but totally diffuse tumoral infiltration. Echographic examination does not reveal calcification as in typical retinoblastoma. Neoplastic cells are demonstrated in anterior chamber paracentesis. Cytologic examination of aqueous humor aspirates may be misinterpreted and should be evaluated carefully. Because of tumor cells seeding, lactate dehydrogenase assay is of diagnostic value, prior to considering definitive therapy. None of the tumors were bilateral. The prognosis after enucleation appears good.