Rhabdomyosarcoma (RMS) of the orbit
Rhabdomyosarcoma “A child with sudden, rapidly progressing unilateral proptosis with an orbital mass → think Rhabdomyosarcoma until proven otherwise.” Malignant tumour of mesenchymal origin showing skeletal muscle differentiation. Most common primary orbital malignancy in children . Epidemiology Accounts for ~4% of all paediatric cancers . 10% of all RMS cases occur in the orbit . Age: usually 5–7 years (rare after adolescence). Sex: slight male predominance . Sites of Origin Orbit (most common intraocular adnexal site). Can arise from extraocular muscles, connective tissue, or blood vessel walls . Histological Subtypes Embryonal (60–70%) – most common in orbit; relatively better prognosis. Alveolar (20–25%) – more aggressive, seen in older children/adolescents. Pleomorphic (rare) – in adults, very aggressive. Botryoid variant – polypoid form under mucosal surfaces. Clinical Features R...