Nephroblastomatosis and wilms tumor: dangerous liaisons

HDNBM (Hyperplastic diffuse nephroblastomatosis) is clinically diagnosed. Histopathology usually cannot differentiate between secondary WT (Wilms tumor) and NBM foci in needle biopsy specimens. This may lead to under treatment or late detection of malignant degeneration (secondary WT) or, on the contrary, overaggressive nephrectomies under the presumed diagnosis of WT in all cases. Inadequate follow-up also leads to late diagnosis of metachronic WT, which is dramatic in cases of anaplastic lineage. In this paper, we review the scarce literature dedicated to HDNBM and summarize two cases treated in on a referral service of Pediatric Urology/Oncology. (author)