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Pathology of Malignant Rhabdoid Tumour of Kidney

 Dr Sampurna Roy MD      June 2015   


Malignant rhabdoid tumour of Kidney (MRTK) is an aggressive renal tumour considered unrelated to Wilms' tumour , and often affecting very young children (mean 11 months).  

Visit: Paediatric Pathology Online  ;  Pediatric Renal Tumours

About 13% of patients develop second midline primitive brain tumours.  

MRTK is associated with hypercalcaemia.

Classical pattern is of "rhabdoid" cells with large eosinophilic central nucleoli and eosinophilic cytoplasmic inclusions which indent the nucleus. There are many other patterns including epithelioid, sclerosing, histiocytoid, lymphomatoid etc.   

The cells co-express vimentin, cytokeratin, often EMA (Epithelial membrane antigen) and occasionally other markers.

Electron microscopy shows the inclusions to be composed of whorls of cytoplasmic filaments with a few included organelles and lipid droplets.

Other renal tumours may simulate MRT or show rhabdoid features. These include Wilms' tumour itself, renal cell carcinoma, cellular examples of mesoblastic nephroma, and medullary cell carcinoma.

Clear Cell Sarcoma of the Kidney , MRTK and Wilms' Tumour with anaplasia are referred to as tumours with unfavourable histology for the purposes of deciding treatment.

Extra-renal rhabdoid tumour is a controversial entity, and has now been described in many sites. Many turn out to be other tumours (neural, epithelial, myoid, mesenchymal, ependymal) when fully investigated.

While renal rhabdoid tumours peak in infancy, reports of extrarenal rhabdoid tumours show a bimodal pattern with a peak in infancy and a second peak in older children tailing into adulthood.

Extra-renal rhabdoid tumours seldom have second intracranial tumours or cause the hypercalcaemia seen with renal rhabdoid tumours.

The relationship between renal and extrarenal rhabdoid tumours is given credence by the demonstration of abnormalities of 22q in CNS, renal and extrarenal malignany rhabdoid tumours.      

 

Further reading:

Rhabdoid tumor of the kidney with spontaneous rupture: case report and review of literature.

P-Akt expression distinguishes two types of malignant rhabdoid tumors

Clinicopathologic study of renal cell carcinoma with rhabdoid features.

Rhabdoid tumors of the kidney in childhood .

Malignant rhabdoid tumor of the kidney in an adult: a case report and review of the literature.  

Mutation Analysis of Human Cytokeratin 8 Gene in Malignant Rhabdoid Tumor: A Possible Association with Intracytoplasmic Inclusion Body Formation.  

High-density single nucleotide polymorphism array analysis in patients with germline deletions of 22q11.2 and malignant rhabdoid tumor.

Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms' Tumor Study Pathology Center.

Malignant rhabdoid tumor: A phenotype? An entity?--A controversy revisited

Establishment and characterization of malignant rhabdoid tumor of the kidney.

Cytologic profile of rhabdoid tumor of the kidney. A report of 3 cases.

Prenatal sonographic features of a rhabdoid tumor of the kidney.

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

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