Orbital Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a highly malignant tumour and one of the most frequent causes of acute exophthalmos in children. It is also one of the few life-threatening diseases that present first to the ophthalmologist. It is the most common soft-tissue sarcoma of the head and neck in childhood, with 10% of all cases occurring in the orbit. RMS has been reported from birth to the seventh decade, with the majority of cases presenting in early childhood. Survival has changed drastically over the years, from 30% in the 1960s to 90% presently, with the advent of new diagnostic and therapeutic modalities.