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July 1981

Wilms' Tumor in a 13-Year-Old Girl With Trisomy 18

Author Affiliations

Long Island Jewish-Hillside Medical Center New Hyde Park, NY

Am J Dis Child. 1981;135(7):665-667. doi:10.1001/archpedi.1981.02130310069024

Trisomy 18, the second most frequent autosomal trisomy, has an incidence ranging from one per 3,500 to one per 7,000 births.1 Although survival beyond infancy is unusual,1 there are a few reports of relatively long-term survivors.2-4 More than 130 different anomalies have been associated with trisomy 18. Neoplasia, however, is a very rare associated finding. The purpose of this article is to describe a 13-year-old girl with trisomy 18 and Wilms' tumor and to stimulate further interest in the association of aneuploidy and neoplasia.

Report of a Case.—The patient was born on Sept 20, 1966, to a 37-year-old mother. The parents were unrelated. The infant was born two weeks prematurely, weighing 2,530 g. She had multiple anomalies including a congenital heart lesion and had delayed physical and psychomotor development from birth.

On Dec 15, 1977, at age 11 years, an abdominal mass was noted and she

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