Orbital malignant peripheral nerve sheath tumors. Treatment with surgical resection and radiation therapy

A series of three patients with primary orbital malignant peripheral nerve sheath tumors (MPNST) is presented. Two of our patients who were treated with surgery and postoperative radiotherapy are free of tumor recurrence. The third patient showed a good response to radiation therapy. While surgical excision remains the mainstay of therapy, our patients demonstrate the usefulness of adjuvant radiation therapy in this condition.