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WHO Classification of Tumours
Malignant rhabdoid tumor


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
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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