Home » What is SARCOMA? » Soft Tissue Sarcoma Reference Guide » Angiosarcoma and Vascular Sarcomas
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Alternative diagnostic termsHemangioendotheliomaMalignant Hemangioendothelioma
Common anatomical locationsMore than 50% of cases arise in cutaneous sites, with the remainder occurring within deep soft tissues, breast, bone, or viscera.
Pathology commentAngiosarcoma is characterized by vasoformative or sheet-like growth, multilayering of endothelial cells, nuclear atypia, increased mitoses, necrosis, CD31, and ERG expression by immunohistochemistry.
SourceAntonescu CR, Thway K, Billings SD. Angiosarcoma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33
For diagnosis or treatmentAny center listed in the Sarcoma Centers Directory »
Patient advocacyAny organization with an established program for patient triage, support, and education. See Patient Support Organizations »
Diagnostic-specific organization:Angiosarcoma Awareness
Clinical trialsSARC Clinical Trials – NoneFind other clinical studies registered on ClinicalTrials.gov
Alternative diagnostic termsNone
Common anatomical locationsEHE most commonly involves somatic soft tissue, lung, and liver, but any site or organ can be affected.
Pathology commentEpithelioid haemangioendothelioma is a malignant vascular neoplasm composed of epithelioid endothelial cells within a distinctive myxohyaline stroma; most cases are characterized by the presence of a WWTR1-CAMTA1 gene fusion.
SourceAntonescu CR, Rubin BP, Doyle LA, Deyrup AT. Epithelioid haemangioendothelioma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2025 Jan 1]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33
Diagnostic-specific organizations:The EHE FoundationICAN – International Cancer Advocacy Network
Alternative diagnostic termsAIDS-associated sarcoma
Common anatomical locationsSkin
Pathology commentKS is uniformly associated with HHV8 infection, and it represents an example of virus-induced vascular proliferation.
SourceAntonescu CR, Thway K, Doyle LA, Mentzel TDW, Grayson W. Kaposi Sarcoma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33
For diagnosis or treatmentAny center listed in the Sarcoma Center Directory »
Common anatomical locationsIntimal sarcoma of the pulmonary circulation mainly involving the proximal vessels: the pulmonary trunk (80%), the right or left pulmonary arteries (50-70%), or both (40%).
Pathology commentThe defining feature is predominantly intraluminal growth with obstruction of the lumen of the vessel origin and seeding of emboli to peripheral organs.
SourceFletcher CDM, Bode-Lesniewska B, Debiec-Rychter M, Tavora F. Intimal Sarcoma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33
Diagnostic-specific organizations:Angiosarcoma Awareness
Alternative diagnostic termsAV Malformation HemangiomaLow-Grade Vascular TumorsBenign and Low-Grade Malignant Vascular Soft Tissue Tumors
Common anatomical locationsPending
Pathology commentPending
SourcePending
Common anatomical locationsPseudomyogenic hemangioendothelioma usually arises on the lower limbs (55% of cases); the upper limbs and trunk are less commonly affected (20% each); tumors rarely occur on the head or neck (5%).
Pathology commentPseudomyogenic hemangioendothelioma is characterized by plump spindles or more rarely, epithelioid cell morphology with vesicular chromatin and eosinophilic cytoplasm; fascicular or sheet-like growth pattern; expression of ERG, FOSB, and keratins (AE1/AE3).
SourceAntonescu CR, Hornick JL, Bovée JVMG, Agaram NP. Pseudomyogenic haemangioendothelioma. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2025 Jan 1]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33
Alternative diagnostic termsGlomus TumorMyopericytomaMyofibromaAngioleiomyoma