Pleomorphic Dermal Sarcoma

Preferred Label : Pleomorphic Dermal Sarcoma;

NCIt synonyms : Malignant Skin Fibrous Histiocytoma; Malignant Fibrous Histiocytoma of Skin; Malignant Fibrous Histiocytoma of the Skin; Cutaneous Unclassified Pleomorphic Sarcoma (Formerly Cutaneous MFH); Cutaneous Unclassified Pleomorphic Sarcoma; Cutaneous Unclassified Pleomorphic Sarcoma (Formerly Cutaneous Malignant Fibrous Histiocytoma); Malignant Cutaneous Fibrous Histiocytoma; Cutaneous Malignant Fibrous Histiocytoma; PDS; Cutaneous Undifferentiated Pleomorphic Sarcoma; Skin Undifferentiated Pleomorphic Sarcoma;

NCIt definition : A rare undifferentiated sarcoma with overlapping features of atypical fibroxanthoma. It occurs in severely sun-damaged skin of elderly patients and usually affects the head and neck region. It manifests with rapidly growing nodules and plaques. Morphologically, it is characterized by dermal proliferation of pleomorphic spindle cells. Epithelioid and multinucleated giant cells may by present. The tumor grows in a fascicular or storiform pattern and invades subcutaneous adipose tissue, skeletal muscle, and fascia. Tumor cell necrosis, perineural invasion, and lymphatic invasion may also be seen. There is increased mitotic activity with atypical mitoses present. Local recurrences occur in 20-30% of cases and metastases in 10-20% of cases.;

Neoplastic status : Malignant;

Details

Origin ID

C5576;

UMLS CUI

C1275254;

Automatic exact mappings (from CISMeF team)
- After-Hours care [MeSH Descriptor]
- Ocular pigment dispersion with or without glaucoma [OMIM Phenotype]
- Pendred syndrome [OMIM Phenotype]
- Pleomorphic dermal sarcoma [ICD-11 Extension code]
- Prostaglandin d2 synthase, brain [OMIM gene]
- Unclassified pleomorphic sarcoma of skin [ICD-11 Subcategory]
- malignant skin fibrous histiocytoma [DO Concept]
- solute carrier family 26 member 4 [ORDO Gene]
CISMeF manual mappings
- Malignant fibrous histiocytoma of skin (disorder) [SNOMED CT concept]
DO Cross reference
- malignant skin fibrous histiocytoma [DO Concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Fascicular Pattern [NCIt concept]
- Lymphatic Invasion [NCIt concept]
- Necrotic Change [NCIt concept]
- Perineural Invasion [NCIt concept]
- Skin Nodule [NCIt concept]
- Skin Plaque [NCIt concept]
- Storiform Growth Pattern [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]
UMLS correspondences (same concept)
- Malignant fibrous histiocytoma of skin (disorder) [SNOMED CT concept]
- malignant skin fibrous histiocytoma [DO Concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Favorable Clinical Outcome [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Spindle Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_associated_disease
- Actinic Keratosis [NCIt concept]
disease_has_finding
- Cellular Pleomorphism [NCIt concept]
- Chronic Sun Exposure [NCIt concept]
- Cutaneous Involvement [NCIt concept]
- Increased Mitotic Activity [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Metastatic/Recurrent Disease [NCIt concept]
- No Identifiable Line of Differentiation [NCIt concept]
- Skin with High Degree of Cumulative Sun Damage [NCIt concept]
- Storiform Growth Pattern [NCIt concept]
- Tissue Differentiation Uncertain [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_may_have_abnormal_cell
- Malignant Epithelioid Cell [NCIt concept]
- Neoplastic Multinucleated Giant Cell [NCIt concept]
- Neoplastic Osteoclast-Like Giant Cell [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Double Minutes [NCIt concept]
- Gain of Chromosome 19 [NCIt concept]
- Gain of Chromosome 1p31 [NCIt concept]
- Gain of Chromosome 7p [NCIt concept]
- Gain of Chromosome 7q32 [NCIt concept]
- Loss of Chromosome 11 [NCIt concept]
- Loss of Chromosome 13 [NCIt concept]
- Loss of Chromosome 16 [NCIt concept]
- Loss of Chromosome 2p [NCIt concept]
- Loss of Chromosome 2q [NCIt concept]
- Supernumerary Circular Chromosome [NCIt concept]
disease_may_have_molecular_abnormality
- NOTCH Family Gene Mutation [NCIt concept]
- TERT Promoter Mutation [NCIt concept]
- TP53 Gene Mutation [NCIt concept]

Keyword's position in hierarchy(ies) :

You can consult :

recommendations
documents concerning education
documents for patients