Malignant Rhabdoid Tumour - Rhabdoid Tumours in Kidney and Brain

Rhabdoid Tumours in Kidney and Brain

Considerable debate has been focused on whether AT/RTs are the same as rhabdoid tumours of the kidney (i.e., just extra-renal MRTs (maligant rhabdoid tumours)). The recent recognition that both CNS atypical teratoid/rhabdoid tumours (AT/RTs) and MRTs have deletions of the INI1 gene in chromosome 22 indicates that rhabdoid tumours of the kidney and brain are identical or closely related entities. Although, the CNS variant tends to have its mutations on Taxon 9 and MRTs elsewhere. This observation is not surprising because rhabdoid tumours at both locations possess similar histologic, clinical, and demographic features. Moreover, 10-15% of patients with MRTs have synchronous or metachronous brain tumours, many of which are second primary malignant rhabdoid tumours. This similarity excludes composite Rhabdoid tumours, which occur mainly in adults.