Haemangioendothelioma and angiosarcoma
- Lesion
Angiosarcoma is the malignant tumour of blood-vessel lining cells. It is rare, spreads early and can follow chemicals, radiation or chronic lymphoedema.
What it is
Malignant endothelial tumours range from intermediate haemangioendotheliomas to the frankly malignant angiosarcoma. Epithelioid haemangioendothelioma grows around medium and large veins, with cells in cords that look epithelial; its course is variable. Angiosarcoma occurs at any site but favours the skin of the scalp and face in older adults, soft tissue, breast and liver. Known causes include hepatic carcinogens (vinyl chloride, arsenic, Thorotrast), prior radiation and chronic lymphoedema (Stewart–Treves syndrome after mastectomy).
Structure
- Gross: red, haemorrhagic, poorly circumscribed masses; skin lesions start as small red nodules and coalesce.
- Well differentiated: anastomosing vascular channels lined by plump, atypical endothelial cells dissecting through tissue.
- Poorly differentiated: solid sheets of spindle or epithelioid cells with many mitoses and necrosis; vascular spaces may be hard to see.
- Epithelioid haemangioendothelioma: cords of rounded cells with cytoplasmic vacuoles (primitive lumens) in a myxoid or hyaline stroma.
Clinical notes
- Because the lesion can mimic a bruise or a benign haemangioma, biopsy of any enlarging purple lesion of the scalp in older adults is important.
Test yourself
Think of the answer, then tap the question to see it.
Which chemical exposures are linked to hepatic angiosarcoma?
Vinyl chloride, arsenic and the old contrast agent Thorotrast.
What is Stewart–Treves syndrome?
Angiosarcoma arising in a chronically lymphoedematous limb, classically the arm after mastectomy with axillary dissection.
Which immunohistochemical markers support endothelial origin?
CD31 and ERG (also FLI1; CD34 is variable).
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version (National Cancer Institute, 2025); Childhood Vascular Tumors Treatment (PDQ®)–Health Professional Version (National Cancer Institute, 2025).
Spotted a mistake?We are always improving for the community. Tell us what is wrong and our team will review it.Report a mistake
Representative model, for educational purposes only.