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# 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.

Updated Nov 12, 2026

## Key points

- Cutaneous angiosarcoma of the scalp and face in older adults looks like a bruise that does not heal.
- Hepatic angiosarcoma: vinyl chloride, arsenic (pesticides) and Thorotrast; long latency.
- Stewart–Treves syndrome: angiosarcoma in a chronically lymphoedematous arm after breast cancer surgery.
- Radiation-associated angiosarcoma: years after breast irradiation; MYC amplification is typical.
- Markers: CD31, ERG and FLI1 (endothelium); CD34 variable.
- Prognosis is poor; many patients have metastases at diagnosis.

## 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.

## Memory aid

Bruise that grows on an old scalp, a lymphoedematous arm, an irradiated breast, a vinyl-chloride liver: think angiosarcoma.

## 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).

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## Connections

## Related 6

- [Chemical carcinogens: initiation, promotion and the table to know](https://durgical.com/en/general-pathology/chemical-carcinogens-initiation-promotion-and-the-table-to-know/)
- [Radiation and cancer: ultraviolet and ionizing](https://durgical.com/en/general-pathology/radiation-and-cancer-ultraviolet-and-ionizing/)
- [Immunohistochemistry: the principle and the markers to know](https://durgical.com/en/general-pathology/immunohistochemistry-the-principle-and-the-markers-to-know/)
- [Kaposi sarcoma](https://durgical.com/en/general-pathology/kaposi-sarcoma/)
- [Lymphangitis and lymphoedema](https://durgical.com/en/general-pathology/lymphangitis-and-lymphoedema/)
- [Rare primary liver cancers](https://durgical.com/en/general-pathology/rare-primary-liver-cancers/)

## Related conditions 3

- [Lymphoedema](https://durgical.com/en/conditions/lymphoedema/)
- [Breast cancer](https://durgical.com/en/conditions/breast-cancer/)
- [Benign liver lesions (haemangioma, focal nodular hyperplasia, adenoma)](https://durgical.com/en/conditions/benign-liver-lesions/)

[← All of general pathology](https://durgical.com/en/general-pathology/)

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](https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq) (National Cancer Institute, 2025); [Childhood Vascular Tumors Treatment (PDQ®)–Health Professional Version](https://www.cancer.gov/types/soft-tissue-sarcoma/hp/child-vascular-tumors-treatment-pdq) (National Cancer Institute, 2025).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/general-pathology/haemangioendothelioma-and-angiosarcoma/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/haemangioendothelioma-and-angiosarcoma/
