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Malignant rhabdoid tumour (MRT) is a very aggressive form of tumour originally described as a variant of Wilms' tumour, which is primarily a kidney tumour that occurs mainly in children. MRT was first described as a variant of Wilms' tumour of the kidney in 1978. MRTs are a rare and highly malignant childhood neoplasm. Later rhabdoid tumours outside the kidney were reported in many tissues including the liver, soft tissue, and the central nervous system. Several cases of primary intracranial MRT have been reported since its recognition as a separate entity in 1978. The term ''rhabdoid'' was used due to its similarity with rhabdomyosarcoma under the light microscope. The exact pathogenesis of MRT is unknown. The cerebellum is the most common location for primary intracerebral MRT (i.e., AT/RT). Biggs et al. were first to report a primary intracranial MRT around 1987. Although the cell of origin is not known, cytogenetic studies have suggested a common genetic basis for rhabdoid tumours regardless of location with abnormalities in chromosome 22 commonly occurring. ==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. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Malignant rhabdoid tumour」の詳細全文を読む スポンサード リンク
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