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Paediatric Pathology Online

Pathology of Clear Cell Sarcoma of the Kidney

Dr Sampurna Roy MD  

 

                                                                                                                      

 

 

Syn: Bone Metastasizing Renal Tumour of Childhood

Like renal rhabdoid tumour, Clear cell sarcoma of the kidney (CCSK) was first recognised as a result of collaborative studies and central pathology review.    

Visit: Paediatric Pathology Online  ;  Pediatric Renal Tumours

Clear cell sarcoma of the kidney (CCSK) occurs rarely before 6 months and is commonest in children aged 1-6 years.

Clear cell sarcoma of the kidney (CCSK ) has no characteristic macroscopic features, although metastasis to bone, particularly the skull is a frequent feature of untreated tumours.

Microscopy typically shows clear cells with vesicular nuclei, a low mitotic rate and a chicken-wire capillary network.

However there are many variants including classic, epithelioid , spindled, sclerosing, myxoid, trabecular, pericytomatous, cystic, storiform, anaplastic and other patterns.  

Some tumours simulate (and possibily may rarely actually show) epithelial differentiation.

Electronmicroscopy shows the clear appearance to be due to extracellular matrix.

Tumour cells are immunoreactive for vimentin.

Clear cell sarcoma of the kidney (CCSK) remains the pediatric renal tumour most frequently misdiagnosed.

Early diagnosis of this tumour is necessary due to its aggressiveness and its ability to cause bone metastases.
 
      

Visit: Clear cell sarcoma & PEComas of the Soft Tissue

 

Further reading:

Recurring translocation (10;17) and deletion (14q) in clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center.

Clear cell sarcoma: a dilemma on pathological staging and clinical management.

Fine-needle aspiration cytology of clear-cell sarcoma of the kidney: study of eight cases.

Clear cell sarcoma of the kidney. A study of 13 cases

Genetic and genetic expression analyses of clear cell sarcoma of the kidney.

A clinicopathological study of clear cell sarcoma of the kidney .

Clear cell sarcoma of the pediatric kidney: detailed description and analysis of variant histologic patterns of a tumor with many faces.

Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis.

Clear cell sarcoma of the kidney: an unusual presentation and review of the literature.

Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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