EC Ophthalmology

Rhabdomyosarcoma (RMS) stands as the predominant malignant mesenchymal tumor among children, constituting 5% of all pediatric malignancies. Its clinical presentation typically includes unilateral proptosis, ptosis, and eyelid swelling, often progressing rapidly. Imaging assumes a pivotal role in guiding diagnosis and ruling out alternative conditions. While MRI remains the cornerstone imaging modality, ultrasonography and CT complementarily contribute to the diagnostic process.

Differential diagnosis encompasses various etiologies of orbital swelling in pediatric patients, such as trauma, infection, and orbital masses. These masses are anatomically classified based on the involved compartment-Intraocular, intra-conal, extra-conal compartments, and the bony orbit. A compartmental approach aids in refining diagnostic orientation by limiting potential differentials.

Definitive diagnosis entails biopsy and histological examination. Staging, according to the Intergroup Rhabdomyosarcoma Study (IRMS) guidelines, entails a comprehensive review of imaging, clinical examination, and residual tumor assessment post-initial surgery, if applicable. Chemotherapy represents the cornerstone of treatment, with radiotherapy and/or surgical debulking serving as adjunct therapeutic options.

This article aims to elucidate the distinctive imaging features of orbital RMS, emphasizing differentiation from potential mimicking conditions. It delineates specific radiological criteria for each diagnosis, offering valuable insights for accurate clinical identification.

 Keywords: Rhabdomyosarcoma; Orbit; MRI; CT; Child

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