Multinodular hyperplastic pannephric nephroblastomatosis with tubular differentiation: A new morphologic variant

Jacinto J. Regalado, Maria Rodriguez, J. Bruce Beckwith

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

We report a case of bilateral nephromegaly delected prenatally with oligohydramnios. Delivered at 35 weeks, this black male infant rapidly developed renal failure, requiring dialysis. He lived 3 1/2 months. The kidneys were three times normal size and diffusely multinodular, with hypoplastic calyces, no corticomedullary demarcation, and no pyramids. Histologically, they revealed hyperplastic embryonal rests composed of tubules and ducts with prominent branching. The nodular rests were intermixed with areas of mature parenchyma, which showed prominent oxalosis. By flow cytometry, rests were diploid, with an S-phase fraction of 25.9%. The proliferation ofembryonal collecting system analogs is attributed to excessively rapid and prolonged branching of the ureteric bud, dating from the fourth gestational month or earlier. This unique case may represent a new morphologic variant of universal nephroblastomatosis.

Original languageEnglish
Pages (from-to)961-972
Number of pages12
JournalFetal and Pediatric Pathology
Volume16
Issue number6
DOIs
StatePublished - Dec 1 1996

Fingerprint

Hyperoxaluria
Oligohydramnios
Diploidy
S Phase
Renal Insufficiency
Dialysis
Flow Cytometry
Kidney

Keywords

  • nephroblastomatosis
  • nephrogenic rest
  • nephromegaly
  • oligohydramnios
  • oxalosis
  • renal dialysis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Multinodular hyperplastic pannephric nephroblastomatosis with tubular differentiation : A new morphologic variant. / Regalado, Jacinto J.; Rodriguez, Maria; Beckwith, J. Bruce.

In: Fetal and Pediatric Pathology, Vol. 16, No. 6, 01.12.1996, p. 961-972.

Research output: Contribution to journalArticle

Regalado, Jacinto J. ; Rodriguez, Maria ; Beckwith, J. Bruce. / Multinodular hyperplastic pannephric nephroblastomatosis with tubular differentiation : A new morphologic variant. In: Fetal and Pediatric Pathology. 1996 ; Vol. 16, No. 6. pp. 961-972.
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