Atypical Teratoid/Rhabdoid Tumor-SHH

Preferred Label : Atypical Teratoid/Rhabdoid Tumor-SHH;

NCIt synonyms : AT/RT-SHH; ATRT-SHH;

NCIt definition : Atypical teratoid/rhabdoid tumor characterized by overexpression of genes in the sonic hedgehog (SHH) pathway (GLI2, BOC, PTCHD2, and MYCN).;

Neoplastic status : Malignant;

Details

Origin ID

C200598;

Disease may have findings
- Aggressive Clinical Course [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Loss of BRG1 Protein Expression [NCIt concept]
- Loss of INI 1 Protein Expression [NCIt concept]
- Necrotic Change [NCIt concept]
Has associated anatomic sites
- Central Nervous System [NCIt concept]
- Nervous System [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
disease_excludes_normal_tissue_origin
- Germinal Layer [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Cell with Large Nucleus and Abundant Eosinophilic Cytoplasm [NCIt concept]
- Malignant Neuroectodermal Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Sonic Hedgehog Pathway Genes Overexpression [NCIt concept]
disease_has_grade
- Grade 4 [NCIt concept]
disease_has_normal_cell_origin
- Embryonic Cell [NCIt concept]
disease_has_normal_tissue_origin
- Embryonic Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Central Nervous System [NCIt concept]
disease_mapped_to_gene
- SMARCA4 Gene [NCIt concept]
- SMARCB1 Gene [NCIt concept]
disease_may_have_abnormal_cell
- Non-Neoplastic Osteoclast-Like Giant Cell [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Loss of Chromosome 22 [NCIt concept]
disease_may_have_molecular_abnormality
- SMARCA4 Gene Mutation [NCIt concept]
- SMARCB1 Gene Mutation [NCIt concept]

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