Ewing's sarcoma in a patient with congenital optic atrophy

Purpose: The authors report Ewing's sarcoma in a patient with congenital op tic atrophy. Congenital optic atrophy is an autosomal dominant disorder wit h variable expression. It manifests itself within the first decade of life and is accompanied by changes in visual acuity. This condition has not been known to cause a pre, disposition toward malignancy. Patient and Methods: In this article, a 16-year-old girl with dominant opti c atrophy and Ewing's sarcoma is described. Results: After tumor resection, the patient was successfully treated for Ew ing's sarcoma with chemotherapy and radiation therapy. Today she has no evi dence of malignancy. However, she continues to have deterioration of her vi sual acuity. Conclusion. This report of cancer in a child with dominant optic atrophy su ggests the possible need for a higher degree of suspicion of malignancy in these patients.