Leiomyosarcoma of the orbit: A case report and review of literature.

Sarcomas account for 1% of adult malignancies, and 80% of them arise from soft tissue. Orbital leiomyosarcoma is an extremely rare tumor with less than 40 cases reported. The most common subsite is the conjunctiva, followed by the eyelid. We present a case of orbital leiomyosarcoma, which presented with progressive prominence of the left globe. The patient was treated with surgery and postoperative radiotherapy. On the last follow-up, the patient is clinically disease-free. A review of the literature on patients with orbital leiomyosarcoma was done, out of which 84% of patients underwent surgery and 42% of patients received radiotherapy. 15% of patients had a childhood history of retinoblastoma (RB). All patients with childhood RB had bilateral disease and a history of childhood radiotherapy for retinoblastoma.