AI Article Synopsis
- Rhabdoid tumor is a rare and aggressive cancer mainly found in infants and young kids, typically appearing in the brain and kidneys, but can also occur in other soft tissues.
- SMARCB1 acts as a tumor suppressor and is often mutated or deleted in these tumors, linking it to a higher risk of rhabdoid tumors and the genetic disorder schwannomatosis.
- Current research suggests these tumors are primarily driven by epigenetic changes rather than specific biological pathway alterations, leading to the exploration of new treatment strategies based on this understanding.
Article Abstract
Rhabdoid tumor is a rare, highly aggressive malignancy that primarily affects infants and young children. These tumors typically arise in the brain and kidney, although extrarenal, non-central nervous system tumors in almost all soft-tissue sites have been described. SMARCB1 is a member of the SWI/SNF chromatin-remodeling complex and functions as a tumor suppressor in the vast majority of rhabdoid tumors. Patients with germline mutations or deletions affecting SMARCB1 are predisposed to the development of rhabdoid tumors, as well as the genetic disorder schwannomatosis. The current hypothesis is that rhabdoid tumors are driven by epigenetic dysregulation, as opposed to the alteration of a specific biologic pathway. The strategies for novel therapeutic approaches based on what is currently known about rhabdoid tumor biology are presented.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6087667 | PMC |
| http://dx.doi.org/10.1615/critrevoncog.2015013566 | DOI Listing |
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