Home > E. Pathology by systems > Urinary system > Kidneys > panlobar nephroblastomatosis
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