Angiosarkooma

Angiosarkooma, also known as angiosarcoma, is a rare malignant tumor that arises from the endothelial cells lining blood vessels or lymphatic channels. It can develop anywhere but most often affects the skin and soft tissues of the head and neck, the breast, and several visceral organs, including the liver. The disease tends to be aggressive, with a high risk of local recurrence and early spread to distant sites. Clinically, it may present as rapidly enlarging, bruise-like lesions, nodules, or masses that may bleed.

Most angiosarcomas are sporadic, but several forms are linked to risk factors. Cutaneous angiosarcoma commonly occurs on the scalp or face of older adults. Angiosarcoma can arise after radiation therapy or in the setting of chronic lymphedema (Stewart-Treves syndrome). Hepatic angiosarcoma has associations with exposure to vinyl chloride, arsenic, or thorium. The exact causes are not fully understood, and the disease is considered biologically diverse with variable behavior.

Diagnosis relies on tissue examination and immunohistochemistry. Biopsies show an infiltrative network of irregular vascular channels lined by atypical endothelial cells. Tumor cells typically express endothelial markers such as CD31 and ERG; CD34 may be variably positive, and other markers can help exclude alternatives. Imaging with MRI or CT helps determine extent and detect metastases.

Treatment is multimodal. For localized disease, surgical excision with wide margins followed by radiotherapy is preferred when feasible. In unresectable or metastatic cases, chemotherapy is used, with paclitaxel commonly employed for cutaneous angiosarcoma and other regimens (such as doxorubicin-based therapies) also in use. Emerging targeted therapies and immunotherapies show potential but lack a standard, proven option. The prognosis is generally poor and depends on factors such as site, size, depth, resection completeness, and metastatic spread.