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Paediatric Pathology Online

Pathology of Nephrogenic Rests

Dr Sampurna Roy MD 

                                                                                                                      

 

 

About 40% of kidneys from patients with Wilms' Tumour contain precursor lesions called nephrogenic rests.

Visit: Paediatric Pathology Online  ;  Pediatric Renal Tumours

Nephroblastomatosis signifies the presence of multiple or diffuse  nephrogenic rests.

Nephrogenic rests are composed of metanephric blastema and immature epithelium and may undergo progression (hyperplastic, adenomatous and neoplastic change) or regression.

There are two main types:

Perilobar: Often multiple, associated with hemihypertrophy, Beckwith-Wiedemann syndrome and familial Wilms' Tumour. Tumours arising are usually epithelial/blastemal.

Intralobar: Often solitary. Associated with aniridia ( complete or partial absence of the iris) , Denys-Drash syndrome and 11p-. Tumours arising are often stromal predominant.

Nephrogenic rests are not themselves malignant, although they might easily be mistaken for Wilms' tumour in a frozen section or small biopsy.

In nephrectomy specimens they occur at the junction of tumour and kidney, beneath the capsule, and in the deep cortex/medulla.

Sample any area of capsular thickening, scar or nodule.

The finding of multiple nephrogenic rests indicates a risk of multiple or bilateral tumour, although in reality the risk of developing a second Wilms' tumour in the remaining kidney is small, perhaps because nephrogenic rests respond to chemotherapy like Wilms' tumour and related entities, and are not seen with Clear Cell Sarcoma of the kidney or Malignant Rhabdoid Tumour.

Further reading:

Nephrogenic rests: their frequency and their fate.

Nephrogenic rests and nephroblastomatosis .

Nephrogenic rests mimicking Wilms' tumor on CT.

Expression of glial cell line-derived neurotrophic factor and neurturin in mature kidney, nephrogenic rests, and nephroblastoma: possible role as differentiating factors.

Clonality and loss of heterozygosity of WT genes are early events in the pathogenesis of nephroblastomas.

Heterotopic nephrogenic rests in the colon and multiple congenital anomalies: possibly related association.

Incidentally detected nephrogenic rests in the setting of congenital obstructive uropathy.

Clinicopathologic features of nephrogenic rests and nephroblastomatosis.

Expression of MIB and BCL-2 in patients with nephrogenic rests with and without associated Wilms' tumors.

Proliferation and maturation indices in nephrogenic rests and Wilms tumor; the emergence of heterogeneity from dormant nodular renal blastema.

Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms' tumor.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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