Ewing's sarcoma in a patient with congenital optic atrophy Journal Article


Authors: Fein-Levy, C.; Gorlick, R.; Meyers, P. A.; Healey, J.; Huvos, A. G.
Article Title: Ewing's sarcoma in a patient with congenital optic atrophy
Abstract: 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.
Keywords: adolescent; treatment outcome; bone neoplasms; cancer surgery; clinical feature; disease course; case report; doxorubicin; cancer risk; multimodality cancer therapy; disease association; genetic association; cyclophosphamide; vincristine; ifosfamide; ewing sarcoma; cancer regression; chromosome translocation; sarcoma, ewing's; radiodiagnosis; autosomal dominant inheritance; ewing's sarcoma; humans; human; female; priority journal; article; congenital optic atrophy; hereditary optic atrophy; optic atrophies, hereditary
Journal Title: Journal of Pediatric Hematology/Oncology
Volume: 20
Issue: 6
ISSN: 1077-4114
Publisher: Lippincott Williams & Wilkins  
Date Published: 1998-11-01
Start Page: 577
End Page: 579
Language: English
DOI: 10.1097/00043426-199811000-00019
PUBMED: 9856684
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 12 December 2016 -- Source: Scopus
Altmetric Score
MSK Authors
  1. Richard G Gorlick
    120 Gorlick
  2. Paul Meyers
    256 Meyers
  3. John H Healey
    405 Healey
  4. Andrew G Huvos
    233 Huvos