Angiosarkom

Angiosarkom, also called angiosarcoma, is a rare and aggressive malignant tumor that arises from endothelial cells lining blood vessels or lymphatic channels. It most often develops in the skin and soft tissues but can occur in the breast, liver, spleen, heart, and other organs. The disease tends to grow quickly and has a high risk of local recurrence and early metastasis, particularly to the lungs and liver.

Clinical presentation varies by location. Cutaneous angiosarcoma commonly appears as bruise-like patches, nodules, or bleeding lesions, often on the scalp or face in elderly patients. Breast angiosarcoma may develop after breast-conserving therapy with radiotherapy or in the setting of chronic lymphedema (Stewart-Treves syndrome).

Risk factors include chronic lymphedema, prior radiotherapy, exposure to vinyl chloride and arsenic, and certain genetic or immunological factors. The tumor may be sporadic or associated with environmental or treatment-related exposures.

Diagnosis is based on biopsy with histopathology showing atypical endothelial cells forming irregular, blood- or lymphatic-filled channels. Immunohistochemical staining commonly shows positivity for endothelial markers such as CD31 and ERG, with variable CD34 expression.

Imaging with MRI or CT assesses local extent; chest CT is used to evaluate metastases. Staging follows soft tissue sarcoma guidelines.

Treatment typically requires a multidisciplinary approach. When feasible, wide surgical excision with negative margins is the mainstay, often followed by radiotherapy in select cases. Systemic chemotherapy is used for unresectable, recurrent, or metastatic disease, with regimens including doxorubicin or paclitaxel; targeted therapies and anti-angiogenic agents are under investigation.

Prognosis is generally poor, with 5-year survival influenced by site and stage; recurrence and distant metastasis are common. See also: soft tissue sarcoma; Kaposi sarcoma.