Abstract
Bilateral renal wedge biopsies of a 2 month old male infant presenting with failure to thrive and uremia showed diffuse renal malformation with nicrocystic tubular dilatation and fetal-type glomeruli, a few showing glomerulosclerosis. Only in retrospective studies were 2 microscopic fuel of nodular renal blastema found. Kidneys removed at 7 years of age after renal transplantation showed a histologically benign monomorphous neoplasm compromised predominantly of microtubular structures with psammoma bodies (diffuse metanephric adenomatosis). Primitive blastema or stromal elements typical of Wilms' tumor were absent. There was no evidence of extrarenal extension.
Nodular renal blastema and derivatives have been described in association with the highly malignant Wilms' tumor. The diffuse lesion in our patient resembles both the focal lesion derived from renal blastema described by Bove et al (Cancer 24, 323, 1969) (metanephric hamartoma) and a diffuse psammomatous Wilms' tumor described by Chatten (Pers. Ped. Path., Vol. III, 1976). The lesion in this patient demonstrated no malignant potential after 7 years and appears to be a benign neoplasm in the Wilms' tumor spectrum.
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Bhathena, D., Bove, K., Wyatt, R. et al. DIFFUSE BILATERAL METANEPHRIC ADEMONA COMPLICATING DIFFUSE RENAL MALFORMATION. Pediatr Res 11, 547 (1977). https://doi.org/10.1203/00006450-197704000-01064
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DOI: https://doi.org/10.1203/00006450-197704000-01064