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Paediatric Pathology Online

Pathology of Wilms' Tumour related Lesions

 Dr Sampurna Roy MD 

 

                                                                                                                      

 

1) Cystic Partially Differentiated Nephroblastoma:

This is a well demarcated lesion composed of cysts often with a hobnail epithelial lining, and with Wilms' tumour-like tissue in their walls.

This lesion is adequately treated by excision alone in children, and is distinguished from cystic Wilms' tumour by the absence of any solid area resembling nephroblastoma.

 

Further reading:

Cystic partially differentiated nephroblastoma.

Partially differentiated cystic nephroblastoma.

Partially differentiated cystic nephroblastoma.

Cystic partially differentiated nephroblastoma and polycystic Wilms tumor: a spectrum of related clinical and pathologic entities.

Cystic partially differentiated nephroblastoma: a clinicopathologic entity in the spectrum of infantile renal neoplasia.

 

2) Metanephric (Nephrogenic) Adenofibroma:  

This tumour arises deep in the kidney.

The two main histologic features are i) spindle cell stroma, ii) nodules of papillary and tubular epithelial elements with psammoma bodies.

The histologic appearance is characterized by a marked proliferation of spindled mesenchymal cells resembling the classical type of congenital mesoblastic nephroma, encasing nodules of embryonal epithelium similar to the hyperplastic nephrogenic rests (nephroblastomatosis) usually associated with Wilms' tumor.

The mesenchymal component consists of a fascicular proliferation of tightly interlaced, uniform, benign-appearing spindled cells that immunostain for vimentin and fibronectin, but not desmin or actin.

The epithelial component consists of discrete islands of blastemal cells that are partially or fully differentiated toward tubular, tubulopapillary, or papillary structures.

Numerous psammoma bodies are present.

Embryonal epithelium immunostains for cytokeratin but not epithelial membrane antigen.

The overall histologic appearance of the mesenchymal and epithelial components is benign.

 

Further reading:

Metanephric adenofibroma in a 10-year-old boy: report of a case and review of the literature.

Metanephric adenofibroma: report of a case and review of the literature.

Nephrogenic adenofibroma. A novel kidney tumor of young people.

The spectrum of metanephric adenofibroma and related lesions: clinicopathologic study of 25 cases from the National Wilms Tumor Study Group Pathology Center.

 

3) Metanephric Adenoma:  

This tumour is composed exclusively of epithelium with characteristic small dark cells arranged in tiny tubules and papillary formations also often with psammoma bodies.

 

Further reading:

Metanephric adenoma vs. Wilms' tumor: a report of 2 cases with diagnosis by fine needle aspiration and cytologic comparisons.

Metanephric neoplasms: the hyperdifferentiated, benign end of the Wilms tumor spectrum?

Metanephric adenoma in an 8-year-old child: case report and review of the literature.

Metanephric adenoma of the kidney.

Metanephric renal adenoma.

Metanephric adenoma. Clinicopathological study of fifty patients.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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