Thelansis

  Rhabdoid Tumor (RT) is a rare and aggressive pediatric soft tissue sarcoma that can arise in various body parts, including the kidney, liver, peripheral nerves, and miscellaneous soft tissues. RT affecting the central nervous system is called Atypical Teratoid Rhabdoid Tumor (ATRT). In most RT cases, there is biallelic inactivation of the SMARCB1 gene, a tumor suppressor gene involved in regulating the expression of the genome. In rare cases, RT is associated with biallelic mutations in the SMARCA4 gene, another SWI/SNF chromatin remodeling complex member. RT commonly occurs in infancy or childhood, with most cases occurring in patients younger than two years old. The first symptoms are usually linked to the compressive effects of a large tumor, such as respiratory distress, abdominal mass, or peripheral nerve palsy. Subcutaneous nodular metastases are a specific feature of neonatal RT cases. The tumor comprises cells with eccentric nuclei, prominent nucleoli, and eosinophilic cyto

Rhabdoid Tumor (RT) is a rare and aggressive pediatric soft tissue sarcoma that can arise in various body parts, including the kidney, liver, peripheral nerves, and miscellaneous soft tissues. RT affecting the central nervous system is referred to as Atypical Teratoid Rhabdoid Tumor (ATRT). In most RT cases, there is biallelic inactivation of the SMARCB1 gene, a tumor suppressor gene involved in regulating the expression of the genome. In rare cases, RT is associated with biallelic mutations in the SMARCA4 gene, another SWI/SNF chromatin remodeling complex member. RT commonly occurs in infancy or childhood, with most cases occurring in patients younger than two years old. The first symptoms are usually linked to the compressive effects of a large tumor, such as respiratory distress, abdominal mass, or peripheral nerve palsy. Subcutaneous nodular metastases are a specific feature of neonatal RT cases. Diagnosis is based on imaging scans and confirmed by biopsy and immunohistochemistry.