Rhabdoid Tumor Overview

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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.
What are the alternative names for Rhabdoid Tumor?
  • Rhabdoid tumor
  • Atypical teratoid rhabdoid tumor
  • BRAIN TUMOR, POSTERIOR FOSSA, OF INFANCY, FAMILIAL
  • Malignant rhabdoid tumor
  • RHABDOID TUMOR PREDISPOSITION SYNDROME 1
  • Rhabdoid sarcoma
  • Rhabdoid tumor predisposition syndrome 2
Who are the top Rhabdoid Tumor Local Doctors?
Elite in Rhabdoid Tumor
Dr. Mrinal M. Gounder
Oncology
Elite in Rhabdoid Tumor
Dr. Mrinal M. Gounder
Oncology

Memorial Solid Tumor Group

1275 York Ave, 
New York, NY 
212-639-2000
Languages Spoken:
English
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Mrinal Gounder is an Oncologist in New York, New York. Dr. Gounder is rated as an Elite provider by MediFind in the treatment of Rhabdoid Tumor. His top areas of expertise are Adult Soft Tissue Sarcoma, Desmoid Tumor, Epithelioid Sarcoma, Liposarcoma, and Liver Embolization.

Mark A. Dickson
Distinguished in Rhabdoid Tumor
Dr. Mark A. Dickson
Hematology Oncology | Hematology | Oncology
Distinguished in Rhabdoid Tumor
Dr. Mark A. Dickson
Hematology Oncology | Hematology | Oncology

Memorial Solid Tumor Group

160 E 53rd St, 
New York, NY 
212-639-2000
Languages Spoken:
English
See accepted insurances
Offers Telehealth

Mark Dickson is a Hematologist Oncology specialist and a Hematologist in New York, New York. Dr. Dickson is rated as a Distinguished provider by MediFind in the treatment of Rhabdoid Tumor. His top areas of expertise are Adult Soft Tissue Sarcoma, Liposarcoma, Undifferentiated Pleomorphic Sarcoma, Gastrointestinal Stromal Tumor, and Endoscopy.

 
 
 
 
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Todd W. Bauer
Distinguished in Rhabdoid Tumor
Dr. Todd W. Bauer
Surgical Oncology
Distinguished in Rhabdoid Tumor
Dr. Todd W. Bauer
Surgical Oncology

University Of Virginia Physicians Group

1215 Lee St, 
Charlottesville, VA 
434-243-6868
Languages Spoken:
English
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Accepting New Patients
Offers Telehealth

Todd Bauer is a Surgical Oncologist in Charlottesville, Virginia. Dr. Bauer is rated as a Distinguished provider by MediFind in the treatment of Rhabdoid Tumor. His top areas of expertise are Cholangiocarcinoma (Bile Duct Cancer), Pancreatic Cancer, Neuroendocrine Tumor, Hepatectomy, and Pancreaticoduodenectomy. Dr. Bauer is currently accepting new patients.

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What are the latest Rhabdoid Tumor Clinical Trials?
TAZNI: A Phase I/II Combination Trial of Tazemetostat With Nivolumab and Ipilimumab for Children With INI1-Negative or SMARCA4-Deficient Tumors
TAZNI: A Phase I/II Combination Trial of Tazemetostat With Nivolumab and Ipilimumab for Children With INI1-Negative or SMARCA4-Deficient Tumors
Enrollment Status: Recruiting
Publish Date: October 03, 2025
Intervention Type: Drug
Study Phase: Phase 1/Phase 2

Summary: This research study involves a combination of three drugs given together as a possible treatment for malignant rhabdoid tumor, atypical teratoid rhabdoid tumor, epithelioid sarcoma, chordoma or other tumors that are deficient in one of two possible proteins, either INI-1 (SMARCB1) or SMARCA4. The names of the study drugs involved in this study are: * Tazemetostat (TAZVERIK) * Nivolumab (OPDIVO) * ...

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B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)
B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)
Who is this study for: Patients with Solid Tumors
Enrollment Status: Recruiting
Publish Date: October 02, 2025
Intervention Type: Drug
Study Drugs: Fludarabine, Cyclophosphamide, MESNA, B7-H3 CAR T cells
Study Phase: Phase 1

Summary: 3CAR is being done to investigate an immunotherapy for patients with solid tumors. It is a Phase I clinical trial evaluating the use of autologous T cells genetically engineered to express B7-H3-CARs for patients ≤ 21 years old, with relapsed/refractory B7-H3+ solid tumors. This study will evaluate the safety and maximum tolerated dose of B7-H3-CAR T cells.The purpose of this study is to find the ...

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Who are the sources who wrote this article ?

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