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 |
|
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 | ||
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 |