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nephroblastomatosis

persistent primitive renal tissue (normally present up to 36 weeks)

potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor

associated with:

hemihypertrophy
sporadic aniridia
pseudohermaphroditism
Klippel-Trenaunay syndrome
Beckwith-Wiedemann syndrome
splenic agenesis with hepatic malformation

findings:

kidneys may be enlarged
deformity of the pelvocalyceal system
US: subtle subcapsular nodules (hypo-/iso-/hyperechoic)
CT: hypodense subcapsular nodules after contrast enhancement

Yong H. Hahn, MD - 2 February 1995
Last updated: 1 October 2013

Related CHORUS documents:

Wilms tumor

Beckwith-Wiedemann syndrome

hemihypertrophy

medullary sponge kidney

aniridia

Drash syndrome

Copyright © 2013, Charles E. Kahn, Jr.