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Soft Tissue Pathology

Pathology of Extrarenal Rhabdoid Tumour

Dr Sampurna Roy MD                          

 

                                                                                                                      

 

Malignant extrarenal rhabdoid tumour (MERT) is a rare soft-tissue neoplasm that occurs in children and young adults.  

Given their highly aggressive nature, early diagnosis of rhabdoid tumours is essential.

Death usually occurs within 2 years.

Metastases occur early to lungs, liver and lymphnodes.

Site: It was first described as as renal tumour, subsequently the tumour was found in soft tissue and in other sites.

Cases have involved numerous extrarenal sites, including the thymus, liver, paravertebral region, central nervous system, heart, prostate, pelvis, chest wall, extremities, as well as soft tissues.

Age:  Most patients are infants or children.

Microscopic features:

1. Round or epitheloid cells with hyaline globular cytoplasmic inclusions and very large nucleoli within vesicular nuclei.

2. Deep and homogeneous acidophilic cytoplasm of the tumor cells is characteristic.

3. Myxoid, pseudoalveolar and hyalinized areas may be seen.

'Rhabdoid inclusions' are present in epithelioid sarcoma, synovial sarcoma, intra-abdominal desmoplastic small cell tumour, rhabdomyosarcoma, melanoma and various types of carcinoma.

Molecular genetic analysis:  Demonstrates deletion or mutation of the hSNF5/INI1 gene, with corresponding reduced expression at the protein level.

Immunohistochemistry: Consistent positivity for cytokeratin, Epithelial Membrane Antigen (EMA) and vimentin. S-100 protein is usually negative.

INI1 antibody immunohistochemistry is useful in confirming the histologic diagnosis of renal or extra-renal rhabdoid tumor, especially for cases with indeterminate histologic features.

Electron microscopy:  Inclusions are paranuclear aggregates of intermediate filaments.

Image and article link: Ettore Marzano, Emilie Lermite, Cinzia Nobili, et al., “Malignant Rhabdoid Tumour of the Liver in the Young Adult: Report of First Two Cases,” HPB Surgery, vol. 2009, Article ID 628206, 4 pages, 2009. doi:10.1155/2009/628206

 

Visit: Rhabdomyomatous Mesenchymal Hamartoma; Rhabdomyoma ;  Cardiac Rhabdomyoma

 

Further reading:

Primary rhabdoid tumor of the brain in an adult.

Urothelial carcinoma with rhabdoid features: report of 6 cases.

Extrarenal malignant rhabdoid tumour of the heel--a case report.

Cutaneous extrarenal rhabdoid tumor with myogenic differentiation.

"Proximal type" epithelioid sarcoma of the vulva: differential diagnosis with other extrarenal rhabdoid tumors.

Skeletal muscle-like and rhabdoid cells in uterine leiomyomas.

Congenital orbital and disseminated extrarenal malignant rhabdoid tumor.

Soft-tissue extrarenal rhabdoid tumor with a unique long-term survival.

Pathologic quiz case. Extrarenal rhabdoid tumor.

Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 


 

 

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