Alveolar Soft Part Sarcoma of the Orbit: A Clinicopathological Report of a Rare Case and Review of the Literature

Background: Alveolar soft part sarcoma (ASPS) of the orbit is a very rare neoplasm with an uncertain histogenesis. The tumor has an indolent clinical course that requires close and long-term follow-up due to late recurrence and metastasis. Surgery is the primary treatment option with adjunct radiotherapy. Case Report: We present the case of a 25-year-old male who presented with a 5-year history of progressive left eye swelling and loss of vision. He underwent left eye exenteration and histology diagnosed ASPS of the orbit. Conclusion: Orbital ASPS is an uncommon soft tissue tumor. Its prognosis is determined by patient’s age, tumor size, and presence of metastasis at the time of diagnosis. Close and long-term monitoring of the patient is required as recurrence is known to occur up to 10 years after treatment.