Ewing's sarcoma in a patient with congenital optic atrophy.

PURPOSE: The authors report Ewing's sarcoma in a patient with congenital optic atrophy. Congenital optic atrophy is an autosomal dominant disorder with 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 predisposition toward malignancy.

PATIENT AND

METHODS

In this article, a 16-year-old girl with dominant optic atrophy and Ewing's sarcoma is described.

RESULTS

After tumor resection, the patient was successfully treated for Ewing's sarcoma with chemotherapy and radiation therapy. Today she has no evidence of malignancy. However, she continues to have deterioration of her visual acuity.

CONCLUSION

This report of cancer in a child with dominant optic atrophy suggests the possible need for a higher degree of suspicion of malignancy in these patients.