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Cancer

Angiosarcoma

What is Angiosarcoma? Causes, Symptoms, Diagnosis, and Treatment

Angiosarcoma is a rare and aggressive form of soft tissue sarcoma that originates in the inner lining (endothelium) of blood vessels or lymphatic vessels. Although it can develop almost anywhere in the body, it most frequently appears on the skin—particularly on the scalp, head, neck, or chest. Less commonly, it may form inside internal organs such as the heart, liver, or spleen. These tumors are malignant and have a high tendency to recur after treatment.

Understanding Angiosarcoma

Because blood vessels and lymphatic vessels exist throughout the body, angiosarcoma can develop in multiple regions. The most frequently affected sites are the scalp and skin of older adults. Internal organ angiosarcomas are rarer but can be more difficult to detect and treat.

This cancer is aggressive and challenging to manage. However, there are several treatment options available today, and early consultation with a healthcare professional can improve outcomes.

Patient undergoing CT scan for diagnosis and monitoring of angiosarcoma
CT scans are used to diagnose and monitor angiosarcoma.

Symptoms of Angiosarcoma

The signs and symptoms vary depending on the area involved:

Cutaneous angiosarcoma (skin, including scalp, face, neck, and chest):

  • Small red or purplish-blue nodules that may enlarge and bleed easily.
  • A bruise-like or purplish area on the skin that can resemble a rash.
  • A non-healing ulcer or lesion that appears to be growing.

Angiosarcoma in internal organs may cause:

  • Persistent fatigue and weakness.
  • Pain localized to the affected organ.
  • Unexplained weight loss.
  • Shortness of breath (angiosarcoma of the heart).
  • Jaundice and upper abdominal pain (angiosarcoma of the liver).
Close-up of a leg with a persistent bruise, highlighting a possible sign of angiosarcoma or another serious condition
See a doctor if you have skin bruises that do not heal or fade over time.

Possible Complications

Angiosarcoma is often diagnosed at an advanced stage, when the cancer has already spread (metastasized). It can affect the skin, liver, heart, and spleen, and metastasize to other organs. Complications may arise from both the disease itself and its treatment. Advanced or metastatic angiosarcoma is difficult to treat, with higher recurrence rates and reduced survival.

When to See a Doctor

Seek medical advice promptly if you notice:

  • New or unexplained bruises or purple skin patches.
  • Non-healing or enlarging skin ulcers.
  • Unexplained pain, fatigue, or weight loss.

Early evaluation increases the chance of timely diagnosis and better outcomes.

Causes of Angiosarcoma

The exact cause remains unclear. Angiosarcoma develops when the DNA of endothelial cells mutates, causing them to grow uncontrollably, evade normal cell death, and form tumors. These abnormal cells can invade surrounding tissue and spread to distant sites.

Illustration of a DNA double helix representing genetic mutations that can transform normal cells into cancer cells
DNA mutations can transform normal cells into cancer cells.

Risk Factors for Angiosarcoma

Although anyone can develop angiosarcoma, certain factors increase the risk:

Age and gender: Most cases occur in adults over 70 years old; men are affected more often than women.

Radiation therapy: Angiosarcoma may develop as a secondary cancer 8–10 years after radiation treatment for another malignancy (commonly breast cancer).

Chemical exposure: Liver angiosarcoma has been linked to long-term exposure to polyvinyl chloride, arsenic, and thorium dioxide; symptoms may not appear for 10–40 years.

Chronic lymphedema: About 5% of angiosarcomas are associated with chronic lymphedema, known as Stewart–Treves syndrome, often after mastectomy with lymph node removal.

Genetic disorders: Around 3% of cases occur in people with inherited conditions such as bilateral retinoblastoma, Maffucci syndrome, neurofibromatosis, or Klippel–Trenaunay syndrome.

Healthcare professional examining a patient's arm for chronic lymphedema which can be associated with angiosarcoma
Chronic lymphedema can be associated with angiosarcoma.

Diagnosis of Angiosarcoma

Doctors start with a physical exam and review of your symptoms and medical history, including any history of radiation therapy or chemical exposure.

Imaging tests may include:

  • CT scan
  • MRI
  • PET-CT

These help determine the tumor’s size, location, and spread. For suspected angiosarcoma of the breast, mammography or breast ultrasound may be ordered; for suspected heart involvement, transesophageal echocardiography may be indicated.

A biopsy—removing a tissue sample for laboratory analysis—is essential to confirm the diagnosis.

Treatment of Angiosarcoma

Medical Therapy:

  • Radiation therapy
  • Chemotherapy
  • Targeted therapy
  • Immunotherapy

Treatment is individualized depending on the patient’s overall health, tumor stage, size, and location.

Surgery:

Surgical removal of the tumor is the most common treatment. Radiation or chemotherapy may be given before surgery to shrink the tumor or after surgery to destroy residual cancer cells.

Lifestyle and Prevention Tips

Although not all risk factors can be prevented (such as inherited conditions or the need for radiation), some steps may reduce your risk:

  • Limit exposure to harmful chemicals at work.
  • Manage lymphedema effectively to reduce the risk of complications.

Self-care during treatment:

  • Stress reduction: Practices like yoga and meditation can help.
  • Rest: Fatigue is common during and after treatment.
  • Stay connected: Seek support from friends, family, and healthcare professionals.
  • Nutrition: Eat a balanced diet and consult a dietitian if eating becomes difficult.
Healthy and nutritious diet including fish, vegetables, grains, and nuts to support angiosarcoma treatment
A healthy and nutritious diet can support the treatment of angiosarcoma.

Dr. Andrew Parker is a board-certified internal medicine physician with over 10 years of clinical experience. He earned his medical degree from the University of California, San Francisco (UCSF), and has worked at leading hospitals including St. Mary’s Medical Center. Dr. Parker specializes in patient education and digital health communication. He now focuses on creating clear, accessible, and evidence-based medical content for the public.

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