Angiosarcoma: Causes, Symptoms, Diagnosis and Treatment — Overview, Diagnosis & Treatment Options | MyMedicPlus

Angiosarcoma is a rare malignant tumor of vascular or lymphatic endothelial cells, accounting for approximately 1-2% of all soft tissue sarcomas. Primary sites include the skin (scalp and face most common), breast, liver, bone, and spleen. Radiation-induced angiosarcoma of the breast and chest wall occurs 5-10 years after radiation therapy, most commonly after breast cancer treatment.

Established causes: chronic lymphedema (Stewart-Treves syndrome: cutaneous angiosarcoma developing in edematous arm after axillary dissection for breast cancer), prior ionizing radiation therapy (5-20 year latency), vinyl chloride and thorotrast exposure (hepatic angiosarcoma), arsenic, and anabolic steroids. Hereditary hemorrhagic telangiectasia (HHT) predisposes to hepatic vascular lesions. Most cases are sporadic.

Cutaneous angiosarcoma: purple-red bruise-like plaque on the scalp or face that bleeds easily and spreads rapidly across multiple skin sites. Post-radiation breast angiosarcoma: red or purple skin nodules in the radiation field. Hepatic angiosarcoma: abdominal pain, hepatomegaly, and spontaneous intra-abdominal hemorrhage. Constitutional symptoms (fever, weight loss) are common. Multifocal lesions frequently occur.

MRI for soft tissue characterization and local extent. CT chest/abdomen/pelvis for staging. Biopsy with immunohistochemistry (CD31, CD34, ERG — endothelial markers) confirms diagnosis. Next-generation sequencing for MYC amplification (radiation-induced angiosarcoma, nearly 100% frequency) and KDR/VEGFR2 mutations. PET/CT for extent of disease and multifocal involvement assessment.

Localized: wide surgical resection with negative margins (challenging due to frequent multifocality). Chemotherapy: weekly paclitaxel has high activity in cutaneous/radiation-induced angiosarcoma (response rate approximately 45-60%) and is often used as neoadjuvant or first-line metastatic treatment. Doxorubicin-ifosfamide for visceral angiosarcoma. Anti-VEGF agents: pazopanib, sorafenib, or bevacizumab for advanced disease. Propranolol (beta-blocker) shows anti-angiosarcoma activity in some series.

Overall prognosis is poor: 5-year OS approximately 20-35%. Cutaneous scalp angiosarcoma is particularly aggressive (5-year OS approximately 15-25%). Radiation-induced breast angiosarcoma: 5-year OS approximately 15-20%. Complete surgical resection offers the best chance of prolonged survival but is often not achievable due to multifocality. Hepatic angiosarcoma: median OS less than 6 months. Paclitaxel has meaningfully improved outcomes for cutaneous disease.