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panlobar nephroblastomatosis

Friday 13 January 2006

Synopsis

- renal anomalies

  • bilateral nephromegaly (renal weight almost 10 times normal)
  • bilaterally large cerebriform kidneys
  • numerous small lobulations containing immature glomeruli admixed with primarily intralobar nephrogenic rests without Wilms tumor (18275253)
  • finely nodular externally, solid
  • cerebriform on cut section
  • diffusely distorted architecture of jumbled lobules
  • hyperplasia of cortical-type tissue with inconspicuous proximal tubules
  • relative hypoplasia of medullary tissue
  • tubulointerstitial dysplasia
  • multicystic renal dysplasia (MRD) (8108294)
  • perilobar nephrogenic rests

Associations

- other renal anomalies

  • multicystic renal dysplasia (MRD) (8108294)
  • tubulointerstitial dysplasia

- oligohydramnios sequence (Potter sequence)

  • small thorax
  • respiratory insufficiency
  • Potter facies
  • hypoplastic lungs (pulmonary hypoplasia)

- fetal ascites

- brain

  • fibrolipomatous hamartomas
    • yellow gelatinous mass in lateral ventricles
    • pseudomyxoid matrix with delicate fibers and focal adipocyte clusters, all confined within choroid plexus

- chromosomal anomalies

Variants

- multinodular hyperplastic pannephric nephroblastomatosis (MHPN)

See also

- nephroblastematoses

References

- Katzman P, Arnold G, Lagoe E, Huff V. Universal nephroblastematosis with bilateral hyperplastic nephromegaly in siblings. Pediatr Dev Pathol. 2008 Feb 14;:1 PMID: 18275253

- Regalado JJ, Rodriguez MM, Beckwith JB. Multinodular hyperplastic pannephric nephroblastomatosis with tubular differentiation: a new morphologic variant. Pediatr Pathol Lab Med. 1996 Nov-Dec;16(6):961-72. PMID: 9025894

- Regalado JJ, Rodriguez MM, Bruce JH, Beckwith JB. Bilateral hyperplastic nephromegaly, nephroblastomatosis, and renal dysplasia in a newborn: a variety of universal nephroblastomatosis. Pediatr Pathol. 1994 May-Jun;14(3):421-32. PMID: 8066001

- Gaulier A, Boccon-Gibod L, Sabatier P, Lucas G. Panlobar nephroblastomatosis with cystic dysplasia: an unusual case with diffuse renal involvement studied by immunohistochemistry. Pediatr Pathol. 1993 Nov-Dec;13(6):741-9. PMID: 8108294