Thoracic SMARCA4-Deficient Undifferentiated Tumor

Preferred Label : Thoracic SMARCA4-Deficient Undifferentiated Tumor;

NCIt synonyms : SMARCA4-Deficient Thoracic Sarcomatoid Tumor; SMARCA4-UT; SMARCA4-Deficient Thoracic Sarcoma;

NCIt definition : An aggressive, high-grade malignant neoplasm characterized by biallelic inactivation of SMARCA4 gene. It affects adults, usually heavy smokers, and involves the thorax (lung, pulmonary hilum, mediastinum, and/or pleura) with or without chest wall invasion. It is composed of sheets of malignant large round to epithelioid cells. Rhabdoid cells may be present. Increased number of mitoses and necrosis are frequently seen. The prognosis is poor.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL1663952;

Details

Origin ID

C183115;

UMLS CUI

C5554190;

Automatic exact mappings (from CISMeF team)
- SMARCA4-deficient sarcoma of thorax [ORDO Disease]
- SMARCA4-deficient thoracic sarcoma [MedDRA LLT]
Excludes anatomical site(s)
- Neck [NCIt concept]
Has associated anatomic sites
- Respiratory System [NCIt concept]
- Thoracic Cavity [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- SMARCA4-deficient thoracic sarcoma [MedDRA LLT]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Epithelioid Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Aggressive Clinical Course [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Necrotic Change [NCIt concept]
disease_has_grade
- High Grade [NCIt concept]
disease_has_molecular_abnormality
- SMARCA4 Gene Inactivation [NCIt concept]
disease_has_primary_anatomic_site
- Thoracic Cavity [NCIt concept]
disease_mapped_to_gene
- SMARCA4 Gene [NCIt concept]
disease_may_have_abnormal_cell
- Rhabdoid Cell [NCIt concept]
pathogenesis_of_disease_involves_gene
- SMARCA4 Gene [NCIt concept]
- SMARCA4 wt Allele [NCIt concept]

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