There have been reported cases of a child having both atypical teratoid rhabdoid tumours in the brain as well as rhabdoid tumours of the kidney. Weeks and associates reported on 111 renal rhabdoid cases of which 13.5% also had a central nervous system malignancy. It has been hypothesized that a germline INI mutation may predispose a child to these tumours. There have been some references in the literature alluding to a new diagnosis called rhabdoid predisposition syndrome related to the gene hSNF5/INI1. These tumours have been associated with mutations in the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1) located on the long arm of chromosome 22 (22q11) and transcription activator BRG1/ATP-dependent chromatin remodeler (SMARCA4) located on the short arm of chromosome 19 (19p13.2). Rhabdoid tumours in kidney and brain Considerable debate has been focused on whether atypical teratoid rhabdoid tumors are the same as rhabdoid tumours of the kidney (i.e., just extrarenal MRTs). Recognition that both CNS atypical teratoid/rhabdoid tumours 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.
types of Mesoblastic nephroma
complications of Mesothelioma
Renal cell carcinoma with t
types of Kidney cancer
complications of Ewing's sarcoma
complications of Teratoma
medical cause of Wandering spleen
symptom of Autosomal recessive polycystic kidney disease