National Cancer Institute Thesaurus

Last uploaded: February 23, 2024
Preferred Name

Rhabdoid Tumor

Synonyms

Rhabdoid Tumor

Rhabdoid sarcoma

rhabdoid tumor

Rhabdoid Sarcoma

Rhabdoid tumor, NOS

Definitions

An aggressive malignant embryonal neoplasm usually occurring during childhood. It is characterized by the presence of large cells with abundant cytoplasm, large eccentric nucleus, and a prominent nucleolus and it is associated with abnormalities of chromosome 22. It can arise from the central nervous system, kidney, and the soft tissues. The prognosis is poor.

ID

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C3808

ALT_DEFINITION

Extremely aggressive malignancies that generally occur in infants and young children. The most common locations are the kidney and central nervous system (CNS), although they can also arise in most soft tissue sites. Approximately two-thirds of patients will present with advanced-stage disease. Rhabdoid tumors of the kidney tend to metastasize to the CNS and lungs. Rhabdoid tumors are associated with mutations of SMARCB1 (INI-1), a tumor suppressor gene.

An aggressive cancer of the central nervous system, kidney, or liver that occurs in very young children."

A malignant tumor of either the central nervous system (CNS) or the kidney. Malignant rhabdoid tumors of the CNS often have an abnormality of chromosome 22. These tumors usually occur in children younger than 2 years.

code

C3808

Concept_In_Subset

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C157711

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C168661

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C90259

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C168656

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C168662

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C168658

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C192842

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C126659

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C165258

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C132009

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C168655

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C168657

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C177537

Contributing_Source

Cellosaurus

GDC

NICHD

DEFINITION

An aggressive malignant embryonal neoplasm usually occurring during childhood. It is characterized by the presence of large cells with abundant cytoplasm, large eccentric nucleus, and a prominent nucleolus and it is associated with abnormalities of chromosome 22. It can arise from the central nervous system, kidney, and the soft tissues. The prognosis is poor.

FULL_SYN

Rhabdoid Tumor

Rhabdoid sarcoma

rhabdoid tumor

Rhabdoid Sarcoma

Rhabdoid tumor, NOS

ICD-O-3_Code

8963/3

Is_Value_For_GDC_Property

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C177621

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C176985

label

Rhabdoid Tumor

Legacy Concept Name

Rhabdoid_Tumor

Maps_To

Malignant rhabdoid tumor

Rhabdoid sarcoma

Rhabdoid tumor, NOS

8963/3

Neoplastic_Status

Malignant

Preferred_Name

Rhabdoid Tumor

prefixIRI

Thesaurus:C3808

Semantic_Type

Neoplastic Process

UMLS_CUI

C0206743

subClassOf

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C3264

Delete Subject Author Type Created
No notes to display