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¹M.D. Karapınar State Hospital, Eye Clinic, Konya/TURKEY
²M.D. Professor, Cukurova University Faculty of Medicine, Department of Ophthalmology, Adana/TURKEY
³M.D., Adana Numune Training and Research Hospital, Eye Clinixc, Adana/TURKEY Malignant peripheral nerve sheath tumors are uncommom aggressive soft tissue sarcomas that originate from peripheral nerves or cells associated with the nerve sheath. The treatment of choice is surgery. Trunk and extremities are the commenest sites involved. Malignant peripheral nerve sheath tumors are rarely encountered in head and neck region. Here, we report a patient presented with a mass at orbit very short history of progression. Histopatholojical examination of biopsy material revealed the diagnosis as malignant mesenchymal tumor. The patient underwent gross total removal of the tumor with adjuvant radiotherapy postoperatively. Keywords : Malignant peripheral nerve sheath tumor, orbit