Abstract
Retinoblastoma is the most common primary intraocular tumor of childhood. Accurate diagnosis at an early stage is important to maximize patient survival, globe salvage, and visual acuity. Management of retinoblastoma is individualized based on the presenting clinical and imaging features of the tumor, and a multidisciplinary team is required to optimize patient outcomes. The neuroradiologist is a key member of the retinoblastoma care team and should be familiar with characteristic diagnostic and prognostic imaging features of this disease. Furthermore, with the adoption of intra-arterial chemotherapy as a standard of care option for globe salvage therapy in many centers, the interventional neuroradiologist may play an active role in retinoblastoma treatment. In this review, we discuss the clinical presentation of retinoblastoma, ophthalmic imaging modalities, neuroradiology imaging features, and current treatment options.
Highlights
Retinoblastoma is diagnosed by physical examination and classified as International Classification of Retinoblastoma groups A through E according to increasing severity
Imaging approaches differ per institution and individual preferences with variations regarding the use of small surface coils or multichannel head coils and imaging at 1.5T or 3T magnet strength
High-resolution MR imaging can identify vitreous seeding as small foci of T2 signal shortening within the vitreous chamber, but clinical examination is more sensitive with a potential role for optical coherence tomography monitoring.[23,24]
Summary
Retinoblastoma is diagnosed by physical examination and classified as International Classification of Retinoblastoma groups A through E according to increasing severity. Retinoblastoma results from a biallelic mutation of the retinoblastoma gene (RB-1) in developing retinal cells, following the 2-hit model of tumor suppressor gene inactivation.[2] In the heritable form, seen in up to 50% of patients, a germline mutation is followed by a second acquired somatic mutation. Retinoblastoma originates in the retina and can display endophytic growth into the vitreous chamber; exophytic growth into the subretinal space; or diffuse, infiltrative growth along the retina. Most tumors demonstrate both endophytic and exophytic growth and can cause retinal detachment as well as vitreous and subretinal tumor seeding
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