Plaque-Like Dermatofibrosarcoma Protuberans

Preferred Label : Plaque-Like Dermatofibrosarcoma Protuberans;

NCIt definition : A rare variant of dermatofibrosarcoma protuberans characterized by plaque-like growth, resembling plaque-like CD34-positive dermal fibroma.;

Neoplastic status : Undetermined;

NCI Metathesaurus CUI : CL1643286;

Details

Origin ID

C177325;

UMLS CUI

C5447697;

Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Integumentary System [NCIt concept]
- Skin [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
disease_excludes_finding
- Well-Circumscribed Lesion [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Cell [NCIt concept]
- Neoplastic Fibroblast [NCIt concept]
- Neoplastic Spindle-Shaped Fibroblast [NCIt concept]
disease_has_finding
- Cutaneous Involvement [NCIt concept]
- Invasive Lesion [NCIt concept]
- Locally Aggressive Lesion [NCIt concept]
- Multinodular Mass [NCIt concept]
- Plaque-Like Lesion [NCIt concept]
- Storiform Growth Pattern [NCIt concept]
disease_has_normal_cell_origin
- Connective and Soft Tissue Cell [NCIt concept]
- Fibroblast [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Dermis [NCIt concept]
- Skin [NCIt concept]
disease_mapped_to_gene
- COL1A1/PDGFB Fusion Gene [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Supernumerary Circular Chromosome [NCIt concept]
- t(17;22)(q22;q13) [NCIt concept]
disease_may_have_molecular_abnormality
- COL1A1-PDGFbeta Fusion Protein Expression [NCIt concept]

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