Learn About Rhabdoid Tumor

What is the definition of Rhabdoid Tumor?
Rhabdoid tumor (RT) is an aggressive pediatric soft tissue sarcoma that arises in the kidney, the liver, the peripheral nerves and all miscellaneous soft-parts throughout the body. RT involving the central nervous system (CNS) is called atypical teratoid Rhabdoid tumor. RT usually occurs in infancy or childhood. In most cases, the first symptoms are linked to the compressive effects of a bulky tumor (such as respiratory distress, abdominal mass, peripheral nerve palsy). In about 90% of cases it is caused by a genetic change in the SMARCB1 gene, which is a tumor suppressor gene. In rare cases, it may be caused by a genetic change in the SMARCA4 gene. No standard care exists for RT, although there are many ongoing studies.
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What are the alternative names for Rhabdoid Tumor?
  • Rhabdoid tumor
  • Atypical teratoid rhabdoid tumor
  • Malignant rhabdoid tumor
  • Rhabdoid sarcoma
  • Rhabdoid tumor predisposition syndrome 2
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What are the latest Rhabdoid Tumor Clinical Trials?
Renal Tumors Classification, Biology, and Banking Study

Summary: This research trial studies kidney tumors in younger patients. Collecting and storing samples of tumor tissue, blood, and urine from patients with cancer to study in the laboratory may help doctors learn more about changes that occur in deoxyribonucleic acid (DNA) and identify biomarkers related to cancer.

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NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-101 (Larotrectinib) in Patients With Tumors Harboring Actionable NTRK Fusions

Summary: This phase II Pediatric MATCH trial studies how well larotrectinib works in treating patients with solid tumors, non-Hodgkin lymphoma, or histiocytic disorders with NTRK fusions that have spread to other places in the body and have come back or do not respond to treatment. Larotrectinib may stop the growth of cancer cells by blocking some of the enzymes needed for cell growth.

Who are the sources who wrote this article ?

Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for Rhabdoid Tumor?
Fetal-onset malignant rhabdoid tumor: a case report.
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