Nephroblastoma (Wilms' tumor) is the most common renal malignancy in c
hildhood. Extrarenal Wilms' tumor is uncommon, and the diagnosis is al
most always postsurgical. The authors report two cases, located in the
sigmoid mesocolon, one of these having an associated horseshoe kidney
. Both were treated with excision followed by chemotherapy. They are n
ow 36 months and 7 months postchemotherapy, respectively, and are symp
tom and recurrence free. Both had favorable histological findings. Ass
ociation with a horseshoe kidney raises an important issue regarding t
he histogenesis of these tumors. The authors believe that there is a n
exus between the fusion of metanephric blastema during the sixth to se
venth week of intrauterine life and the ''ectopic'' metanephric blaste
ma cells that may give rise to extrarenal Wilms' tumor. Association wi
th a horseshoe kidney with an extrarenal Wilms' tumor has been reporte
d on five previous occasions. The authors closely examine the link bet
ween the two. J Pediatr Surg 33:935-937. Copyright (C) 1998 by W.B. Sa
unders Company.