Angiosarcoma

What it is

Overview

Angiosarcoma is a rare and aggressive malignancy arising from the endothelial cells that line blood or lymphatic vessels. It can develop anywhere in the body but most commonly occurs in the skin (especially the scalp and face of elderly men), the breast (often after radiation), and the liver. It is notorious for being difficult to diagnose early and for its poor prognosis even with treatment.

Biology

How It Develops

Angiosarcoma arises from endothelial cells with mutations in KDR, TP53, MYC amplification (in radiation-induced cases), and complex genomic rearrangements. Secondary angiosarcoma — occurring after radiation therapy or in areas of chronic lymphedema — is increasingly recognized, particularly breast angiosarcoma after breast-conserving surgery and radiation.

Warning signs

Symptoms

• —A skin lesion that looks like a bruise that won't heal
• —Purple, red, or bluish nodule or plaque on skin
• —Swelling in the affected area
• —Abdominal pain (hepatic angiosarcoma)
• —Breast mass or skin changes

Detection

Diagnosis Methods

• —Biopsy with immunohistochemistry (CD31, CD34, ERG markers)
• —MRI and CT for extent of disease
• —PET scan for metastases

Medical care

Treatment Options

• —Wide surgical excision (often difficult to achieve clear margins)
• —Radiation therapy
• —Chemotherapy (paclitaxel is the most active agent)
• —Anti-angiogenic targeted therapy (bevacizumab)
• —Clinical trials

Data

Statistics

Prevention

Risk Factors

• —Prior radiation therapy
• —Chronic lymphedema (Stewart-Treves syndrome)
• —Chemical exposure (vinyl chloride — for hepatic angiosarcoma)
• —Advanced age

Further reading

Resources