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.