8963/3 Rhabdoid sarcoma


Related terms

Malignant rhabdoid tumor

Definitions

Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

Malignant rhabdoid tumour is an aggressive embryonal neoplasm usually manifesting during childhood. It is histologically characterized by the presence of large cells with abundant cytoplasm, eccentric nucleus, and a prominent nucleolus and is associated with mutational loss of expression of the INI1 gene. The prognosis is poor. Common sites of involvement are kidney (MRTK), brain (AT/RT), soft tissues and liver 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Malignant rhabdoid tumour (RT) of the liver
Malignant rhabdoid tumour (RT) primary to the liver is a rare and highly aggressive tumour that is characterized by a diffuse growth of undifferentiated cells with socalled rhabdoid features and a distinct molecular change in some cases. The concept of rhaboid tumour or of tumours with rhabdoid features is based on the observation of intracellular filamentous aggregates manifest as distinct paranuclear spherical inclusions
2
Click to access Pubmed
Gonzalez-Crussi F, Goldschmidt RA, Hsueh W, Trujillo YP (1982)
Infantile sarcoma with intracytoplasmic filamentous inclusions: distinctive tumor of possible histiocytic origin.
Cancer 49: 2365-75



, rendering the cell asymmetrical and somewhat resembling a rhabdomyoblast. However, the cell lineage involved in RT has nothing to do with rhabdomyosarcoma, and the cell type involved is still unknown. RT is a well-known entity in the kidney, but primary hepatic RT is a rare high-risk lesion of still unknown frequency 3
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Hunt SJ, Anderson WD (1990)
Malignant rhabdoid tumor of the liver. A distinct clinicopathologic entity.
Am J Clin Pathol 94: 645-8



4
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Parham DM, Weeks DA, Beckwith JB (1994)
The clinicopathologic spectrum of putative extrarenal rhabdoid tumors. An analysis of 42 cases studied with immunohistochemistry or electron microscopy.
Am J Surg Pathol 18: 1010-29



5
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

Malignant rhabdoid tumour is an aggressive embryonal neoplasm usually manifesting during childhood. It is histologically characterized by the presence of large cells with abundant cytoplasm, eccentric nucleus, and a prominent nucleolus and is associated with mutational loss of expression of the INI1 gene. The prognosis is poor. Common sites of involvement are kidney (MRTK), brain (AT/RT), soft tissues and liver
6
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

Malignant rhabdoid tumour is an aggressive embryonal neoplasm usually manifesting during childhood. It is histologically characterized by the presence of large cells with abundant cytoplasm, eccentric nucleus, and a prominent nucleolus and is associated with mutational loss of expression of the INI1 gene. The prognosis is poor. Common sites of involvement are kidney (MRTK), brain (AT/RT), soft tissues and liver
7
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

Malignant rhabdoid tumour is an aggressive embryonal neoplasm usually manifesting during childhood. It is histologically characterized by the presence of large cells with abundant cytoplasm, eccentric nucleus, and a prominent nucleolus and is associated with mutational loss of expression of the INI1 gene. The prognosis is poor. Common sites of involvement are kidney (MRTK), brain (AT/RT), soft tissues and liver
8
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.