Bilateral nephroblastomatosis and Klippel trenaunay syndrome

Abstract

An infant with Klippel Trenaunay Syndrome (KTS), a disorder of embryonic development characterized by hemangiomas and hypertrophy of lower extremities, had bilateral nephroblastomatosis diagnosed at 1 year of age. Although the natural course of these tumors is uncertain, lesions of this kind have been suggested as the source of Wilms' tumor. In addition, foci of primitive cells suggested malignant potential. The more extensively involved kidney was removed and the contralateral lesion was treated by irradiation, actinomycin D, and vincristine, resulting in disappearance of the lesions 6 months later. The association of nephroblastic tumors with soft tissue growth disorders is extended to include KTS, and the possible relationship of nephroblastomatosis to Wilms' tumor is discussed. Copyright © 1974 American Cancer Society