Retinoblastoma and beyond: pediatric orbital mass lesions

Abstract

Various space occupying lesions can arise in the orbit, ranging from developmental anomalies to malignancies, and many of the diseases occurring in children are different from the pathologies in the adult population. As the clinical presentation is frequently nonspecific, radiologic evaluation is essential for lesion detection and characterization as well as patient management. While orbital masses may in some cases involve multiple compartments, a simple compartmental approach is the key for the diagnosis on imaging studies, and MRI is the modality of choice. This pictorial review presents the most common and characteristic non-emergent pediatric orbital lesions, stressing their MRI and CT appearances, including specific differentiating features. The lesions are subdivided into 4 compartments: intraocular, intraconal, extraconal, and orbital walls. Retinoblastoma, Coats disease and persistent fetal vasculature; optic pathway glioma and lymphovascular malformations; rhabdomyosarcoma, infantile hemangioma, neurofibroma and lymphoma; neuroblastoma, leukemia/myeloid sarcoma, Langerhans cell histiocytosis and dermoid are reviewed in their respective compartments.