Major Salivary Gland Adamantinoma-Like Ewing Sarcoma

Preferred Label : Major Salivary Gland Adamantinoma-Like Ewing Sarcoma;

NCIt synonyms : Salivary Gland Adamantinoma-Like Ewing Sarcoma; Major Salivary Gland ALES; Salivary Gland ALES;

NCIt definition : An exceedingly rare variant of Ewing sarcoma with epithelial differentiation that resembles carcinoma and arises from the major salivary glands. Morphologically, it is characterized by the presence of nests of basaloid cells, tumor cell palisading, myxoid or hyalinized stroma, focal keratin pearl formation, and high-grade features with minimal pleomorphism.;

Neoplastic status : Malignant;

Détails

Origin ID

C213630;

Disease excludes abnormal cell
- Malignant Epithelial Cell [NCIt concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Fever [NCIt concept]
- Fibrotic Stroma Formation [NCIt concept]
- Hemorrhagic Change [NCIt concept]
- Homer Wright Rosette Formation [NCIt concept]
- Hyalinized Stroma Formation [NCIt concept]
- Myxoid Stroma Formation [NCIt concept]
- Necrotic Change [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
- Maxillofacial Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Head and Neck [NCIt concept]
- Major Salivary Gland [NCIt concept]
- Salivary Gland [NCIt concept]
- Soft Tissue [NCIt concept]
Has cytogenetic abnormalities
- EWSR1 Gene Rearrangement [NCIt concept]
disease_excludes_cytogenetic_abnormality
- CIC Gene Rearrangement [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
- Neuroectodermal Differentiation [NCIt concept]
disease_excludes_molecular_abnormality
- EWSR1-NFATC2 Fusion Protein Expression [NCIt concept]
- EWSR1-PATZ1 Fusion Protein Expression [NCIt concept]
- FUS-NFATC2 Fusion Protein Expression [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
- Germinal Layer [NCIt concept]
disease_has_abnormal_cell
- Malignant Basaloid Cell [NCIt concept]
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Primitive Round to Oval Cell [NCIt concept]
disease_has_finding
- Epithelial Differentiation [NCIt concept]
- Extraosseous Lesion [NCIt concept]
- High Grade Lesion [NCIt concept]
- Keratin Pearl Formation [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Metastatic/Recurrent Disease [NCIt concept]
- Peripheral Nuclear Palisading [NCIt concept]
- Tissue Differentiation Uncertain [NCIt concept]
disease_has_grade
- High Grade [NCIt concept]
disease_has_normal_cell_origin
- Embryonic Cell [NCIt concept]
disease_has_normal_tissue_origin
- Embryonic Tissue [NCIt concept]
- Neural Crest [NCIt concept]
disease_has_primary_anatomic_site
- Connective and Soft Tissue [NCIt concept]
- Head and Neck [NCIt concept]
- Major Salivary Gland [NCIt concept]
- Soft Tissue [NCIt concept]
disease_mapped_to_gene
- EWSR1 Gene [NCIt concept]
- EWSR1/ERG Fusion Gene [NCIt concept]
- EWSR1/ETV1 Fusion Gene [NCIt concept]
- EWSR1/ETV4 Fusion Gene [NCIt concept]
- EWSR1/FEV Fusion Gene [NCIt concept]
- EWSR1/FLI1 Fusion Gene [NCIt concept]
- FUS/ERG Fusion Gene [NCIt concept]
disease_may_have_abnormal_cell
- Malignant Small Round Cell [NCIt concept]
disease_may_have_associated_disease
- Anemia [NCIt concept]
disease_may_have_cytogenetic_abnormality
- t(11;22)(q24;q12) [NCIt concept]
- t(16;21)(p11;q22) [NCIt concept]
- t(17;22)(q21;q12) [NCIt concept]
- t(1;22)(p36.1;q12) [NCIt concept]
- t(21;22)(q22;q12) [NCIt concept]
- t(2;22)(q33;q12) [NCIt concept]
- t(7;22)(p22;q12) [NCIt concept]
disease_may_have_molecular_abnormality
- EWSR1-ERG Fusion Protein Expression [NCIt concept]
- EWSR1-ETV1 Fusion Protein Expression [NCIt concept]
- EWSR1-ETV4 Fusion Protein Expression [NCIt concept]
- EWSR1-FEV Fusion Protein Expression [NCIt concept]
- EWSR1-FLI1 Fusion Protein Expression [NCIt concept]
- FUS-ERG Fusion Protein Expression [NCIt concept]

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