Bilateral hyperplastic nephromegaly, nephroblastomatosis, and renal dysplasia in a newborn: A variety of universal nephroblastomatosis

J. J. Regalado, M. M. Rodriguez, J. H. Bruce, J. B. Beckwith

Research output: Contribution to journalArticlepeer-review

9 Scopus citations

Abstract

A preterm boy was born at 34 weeks. Prenatal ultrasonography showed oligohydramnios, fetal ascites, large kidneys, and small thorax. He died 21 h after birth of respiratory insufficiency. Autopsy revealed Potter's-like facies, hypoplastic lungs, ascites, and bilateral nephromegaly (renal weight almost 10 times normal). The kidneys were finely nodular externally, solid, and cerebriform on cut section. Histologically, they showed a diffusely distorted architecture of jumbled lobules, hyperplasia of cortical-type tissue with inconspicuous proximal tubules, relative hypoplasia of medullary tissue, tubulointerstitial dysplasia, and perilobar nephrogenic rests. The renal features represent a variety of the universal or panlobar (also called pancortical or infantile) type of nephroblastomatosis. To our knowledge, this is only the third such case reported. In the brain, each lateral ventricle contained a yellow gelatinous mass. Histologically, the masses consisted of a pseudomyxoid matrix with delicate fibers and focal adipocyte clusters, all confined within choroid plexus. We consider these lesions fibrolipomatous hamartomas..

Original languageEnglish (US)
Pages (from-to)421-432
Number of pages12
JournalFetal and Pediatric Pathology
Volume14
Issue number3
DOIs
StatePublished - Jan 1 1994

Keywords

  • Choroid plexus hamartoma
  • Nephroblastomatosis
  • Nephrogenic rests
  • Nephromegaly
  • Oligohydramnios
  • Renal dysplasia

ASJC Scopus subject areas

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

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