Pseudomyogenic Hemangioendothelioma

Preferred Label : Pseudomyogenic Hemangioendothelioma;

NCIt synonyms : Epithelioid Sarcoma-Like Hemangioendothelioma; Pseudomyogenic (Epithelioid Sarcoma-Like) Hemangioendothelioma;

NCIt definition : An intermediate, rarely metastasizing blood vessel neoplasm that more frequently affects young adult males and usually arises in the lower limbs. In approximately half of the affected patients the tumor is painful and in two-thirds of the patients the tumor is multifocal. Morphologically it is characterized by the presence of sheets and fascicles of spindle cells with abundant eosinophilic cytoplasm and vesicular nuclei. Cytologic atypia is usually mild. Approximately 60% of the patients develop local recurrences or additional tumors in the same anatomic region.;

Neoplastic status : Undetermined;

Details

Origin ID

C121668;

UMLS CUI

C3840252;

Currated CISMeF NLP mapping
- Pseudomyogenic (epithelioid sarcoma-like) haemangioendothelioma [ICD-11 Extension code]
- Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (morphologic abnormality) [SNOMED CT concept]
Disease may have findings
- Multifocal Lesion [NCIt concept]
- Pain [NCIt concept]
- Skin Nodule [NCIt concept]
- Subcutaneous Nodule [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Cardiovascular System [NCIt concept]
- Connective and Soft Tissue [NCIt concept]
- Vascular System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (morphologic abnormality) [SNOMED CT concept]
concept_is_in_subset
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
disease_has_abnormal_cell
- Epithelioid Endothelial Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Endothelial Cell [NCIt concept]
- Spindle Endothelial Cell [NCIt concept]
disease_has_finding
- Locally Aggressive Lesion [NCIt concept]
- Rarely Metastasizing Lesion [NCIt concept]
- Vascular Channel Formation [NCIt concept]
disease_has_normal_cell_origin
- Endothelial Cell [NCIt concept]
disease_has_normal_tissue_origin
- Blood Vessel Endothelium [NCIt concept]
- Soft Tissue [NCIt concept]
- Vascular Endothelium [NCIt concept]
disease_has_primary_anatomic_site
- Blood Vessel [NCIt concept]
- Vascular System [NCIt concept]
disease_mapped_to_gene
- ACTB/FOSB Fusion Gene [NCIt concept]
- SERPINE1/FOSB Fusion Gene [NCIt concept]
disease_may_have_cytogenetic_abnormality
- t(7;19)(q22;q13) [NCIt concept]
pathogenesis_of_disease_involves_gene
- ACTB/FOSB Fusion Gene [NCIt concept]
- SERPINE1/FOSB Fusion Gene [NCIt concept]

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