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 mutation in the SMARCB1 gene, which is a tumor suppressor gene. In rare cases, it may be caused by a mutation in the SMARCA4 gene. No standard care exists for RT, although there are many ongoing studies. Treatment includes resection of the tumor mass and chemotherapy and radiotherapy. Because atypical teratoid rhabdoid tumors and rhabdoid tumors of the kidney have the same gene mutation and similar biopsy findings, they are now considered identical or closely related entities. Also, 10-15% of patients with malignant rhabdoid tumors have brain tumors.

What are the alternative names for Rhabdoid Tumor?

  • Rhabdoid sarcoma
  • Malignant rhabdoid tumor
  • Rhabdoid tumor predisposition syndrome 2
  • Atypical teratoid rhabdoid tumor
  • Condition: Metastatic Atypical Teratoid/Rhabdoid Tumors
  • Journal: Pediatric neurology
  • Treatment Used: Autologous Hematopoietic Cell Rescue, Radiation Therapy, and Intrathecal Chemotherapy
  • Number of Patients: 130
  • Published —
This study investigated various treatment modalities in patients with metastatic atypical teratoid/rhabdoid tumors.
  • Condition: Atypical Teratoid/Rhabdoid Tumor (AT/RT)
  • Journal: Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • Treatment Used: Intensive Postoperative Chemotherapy and Focal Radiation
  • Number of Patients: 65
  • Published —
This study examined the effectiveness and safety of intensive postoperative (after surgery) chemotherapy and focal radiation (delivers radiation from multiple directions in a very targeted way) to treat atypical teratoid/rhabdoid tumor (rare and fast-growing cancerous tumor of the brain and spinal cord; AT/RT).
Clinical Trial
  • Status: Recruiting
  • Phase: Phase 1
  • Intervention Type: Biological, Drug, Procedure
  • Participants: 20
  • Start Date: January 14, 2021
A Pilot Study of Allogeneic Hematopoietic Cell Transplantation for Patients With High Grade Central Nervous System Malignancies
Clinical Trial
  • Status: Recruiting
  • Phase: Phase 2
  • Intervention Type: Drug
  • Participants: 49
  • Start Date: September 14, 2020
NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations