Alveolar soft part sarcoma (ASPS) is a rare malignancy that accounts for less than 1% of all soft tissue tumours. In a localized disease, it has a reported 5-year disease-free survival of 71% and decreases to 20% in patients who present with metastases. Although it has been more than half a decade since this condition has been reported, there is still limited knowledge available regarding this condition. Since this may present similar to other types of soft tissue tumours in the orbit, histologic evaluation becomes vital. Local surgical excision appears to provide the best chance of controlling the disease involving the orbit.
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