Rhabdomyosarcoma (RMS) is a highly malignant tumor and is 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 1960's to 90% presently, with the advent of new diagnostic and therapeutic modalities. The purpose of this review is to provide a general overview of primary orbital RMS derived from a literature search of material published over the last 10 years, as well as to present two representative cases of patients that have been managed at our institute.

Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with primary exenteration only one of whom was alive 3 years after diagnosis. Radical surgical excision is thus the first treatment of choice for primary orbital epithelioid sarcoma. The authors present a patient with primary orbital epithelioid sarcoma who refused exenteration. Surgical debulking followed by local brachytherapy was performed. The patient remains tumor free 5 years after diagnosis. The literature remains limited regarding treatment options for primary orbital epithelioid sarcoma. However, based on reported cases and this case, the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable treatment when treating primary orbital epithelioid sarcoma.