EXPERIENCE WITH 6 CHILDREN WITH FETAL RHABDOMYOMATOUS NEPHROBLASTOMA - REVIEW OF THE CLINICAL, BIOLOGICAL, AND PATHOLOGICAL FEATURES

Background. Fetal rhabdomyomatous nephroblastoma (FRN) is a rare varia nt of Wilms tumor. Materials and Results. One hundred and thirty two c hildren with kidney tumors were seen al our hospital from 1985 to 1993 . Among them were 6 (4.5%) who had FRNs. Five were boys aged 8 months to 3 years; the girl was 17 months old. Three of the four with unilate ral disease had tumors so large that they were considered unresectable at diagnosis. Five received lire-operative chemotherapy and three als o received pre-operative radiation therapy. None of the tumors respond ed. Both patients with bilateral tumors died of progressive disease. T hree oi the four patients with unilateral disease followed for at leas t. one year are alive for 1 to 10 years after diagnosis. Conclusions. FRN should be in the differential diagnosis of huge kidney tumors in c hildren, and preoperative therapies escalated with caution since FRN i s not responsive to treatment used for classic Wilms tumor. (C) 1998 W iley-Liss, Inc.