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Malignant Rhabdoid Tumour (MRT) of kidney is an aggresive sarcomatous neoplasm with a histology distinct from Wilms' tumour. In a review of 111 cases of MRT of kidney by the National Wilms' Tumor Study (Weeks, 1989) the median age at diagnosis was 11
months (range 0 - 106 months) and the male:female ratio was 1.5:1. Treatment for MRT of kidney is often similar to that for Wilms' tumour (MRT starting in the brain or other non-renal sites have different treatments). A common cytogenetic feature of MRT is
deletion of material from chromosome 22q11.2 including the SMARCB1 (SNF5/INI1) gene.
See also Wilms' Tumour
See also SMARCB1 gene (SNF5 / INI1)
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