Angiosarcoma

Angiosarcoma is a rare and aggressive malignant tumor that arises from the endothelial cells lining blood vessels or lymphatic vessels. It belongs to the group of soft tissue sarcomas and can occur in virtually any part of the body, though it most commonly affects the skin and superficial soft tissues (particularly of the head and scalp in elderly individuals), the breast, the liver, the spleen, and deep soft tissues. Angiosarcoma of the skin may present as bruise-like lesions, purplish-red nodules, or plaques that can ulcerate and bleed. Visceral angiosarcomas may cause organ-specific symptoms such as abdominal pain, liver failure, or internal hemorrhage. The disease tends to grow rapidly and has a high rate of local recurrence and distant metastasis, most commonly to the lungs, liver, lymph nodes, and bone. Known risk factors include chronic lymphedema (Stewart-Treves syndrome), prior radiation therapy (radiation-associated angiosarcoma, particularly of the breast), and exposure to certain chemicals such as vinyl chloride, thorium dioxide (Thorotrast), and arsenic. Hepatic angiosarcoma has been specifically linked to vinyl chloride and Thorotrast exposure. Most cases, however, arise sporadically without an identifiable predisposing factor. The disease predominantly affects older adults, with a median age of diagnosis typically in the sixth to seventh decade of life, though it can occur at any age. Treatment of angiosarcoma is challenging due to its aggressive biology and tendency for multifocal spread. The primary treatment for localized disease is wide surgical excision, often combined with radiation therapy to reduce local recurrence. Chemotherapy, typically with taxanes (paclitaxel, docetaxel) or anthracyclines (doxorubicin), is used for advanced or metastatic disease and may also be employed in the neoadjuvant or adjuvant setting. Targeted therapies, including anti-angiogenic agents such as bevacizumab and pazopanib, have shown some activity. Immunotherapy with checkpoint inhibitors is being investigated. Despite multimodal treatment, the overall prognosis remains poor, with five-year survival rates generally ranging from 10% to 35% depending on the site and stage at diagnosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Angiosarcoma