Bilateral Hyperplastic Nephromegaly, Nephroblastomatosis, and Renal Dysplasia in a Newborn: A Variety of Universal Nephroblastomatosis

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.

Bilateral Renal Dysplasia, Nephroblastomatosis, and Bronchial Stenosis. A New Syndrome?

“…The case published in 1994 contained a more classical renal blastemal comprising confluent perilobar nephrogenic rests with an associated renal dysplasia. On the other hand, the 1996 case had tubular differentiation resembling the epithelial component of some Wilms tumors [10,11]. Lastly, a more recent report on two siblings with bilateral nephromegaly and autopsy proven universal NB, suggested an autosomal recessive mode of inheritance [12].…”

“…The autopsy revealed generalized visceromegaly, islet cell hyperplasia and hypertrophy, and nephroblastomatosis. Several small series and single case reports of NB detected at autopsy have followed over the course of the next three decades [8][9][10][11]. A series by De Chadarevian et al described four children with nephromegaly detected on physical examination and confirmed by imaging.…”

Multinodular Hyperplastic Pannephric Nephroblastomatosis with Tubular Differentiation: A New Morphologic Variant

“…This unique case of N B does not fit neatly into the classification scheme proposed by Beckwith et al [ 11. It comes closest to universal NB, the rarest type, ofwhich only a handful of cases are reported [5,. Beckwith et al…”

“…It includes diffuse hyperplastic perilobar NB, previously called superficial diffuse or massive NB [ 3 , 41, and the very rare universal NB [5]. We present a new and apparently unique morphologic variant of pure, clinically striking N B in a neonate.…”

Bilateral universal nephroblastomatosis in an 8-month-old infant treated with chemotherapy

“…The variant of universal nephroblastomatosis is very uncommon. All reported cases of universal nephroblastomatosis have occurred in infants younger than 4 months old [1][2][3][4][5][6], almost all were bilateral. Most of these cases were associated with other congenital abnormalities.…”