Overview

Angiosarcoma is a rare type of cancer that forms in the lining of the blood vessels and lymph vessels. The lymph vessels are part of the immune system. The lymph vessels collect bacteria, viruses and waste products from the body and dispose of them.

This type of cancer can occur any place in the body. But it most often occurs in the skin on the head and neck. Rarely, it may form in the skin on other parts of the body, such as the breast. Or it may form in deeper tissue, such as the liver and the heart. Angiosarcoma can occur in areas that were treated with radiation therapy in the past.

Angiosarcoma that affects the skin

Angiosarcoma of the skin

Angiosarcoma is cancer that forms in the lining of blood vessels and lymph vessels. It often affects the skin and may appear as a bruise-like lesion that grows over time.

Treatment depends on where the cancer is located. Treatments may include surgery, radiation therapy and chemotherapy.


Symptoms

Angiosarcoma signs and symptoms may vary based on where the cancer occurs.

Angiosarcoma that affects the skin

Most often, angiosarcoma occurs in the skin on the head and neck. It often happens on the scalp. Symptoms of this form of angiosarcoma include:

  • A raised area of skin that looks like a bruise
  • A bruise-like lesion that grows larger over time
  • A lesion that may bleed when scratched or bumped
  • Swelling in the skin around the lesion

Angiosarcoma that affects organs

When angiosarcoma affects organs, such as the liver or the heart, it often causes pain. Other symptoms depend on the location of the angiosarcoma.


When to see a doctor

Make an appointment with your health care provider if you have any persistent symptoms that worry you.


Causes

It's not clear what causes most angiosarcomas. Researchers have identified factors that may increase the risk of the disease.

Angiosarcoma happens when cells in the lining of a blood vessel or lymph vessel develop changes in their DNA. A cell's DNA contains the instructions that tell the cell what to do. The changes, which doctors call mutations, tell the cells to multiply quickly. The changes cause the cells to keep living when healthy cells would die.

The result is a buildup of cancer cells that can grow beyond the blood vessel or lymph vessel. The cancer cells can invade and destroy healthy body tissue. In time, cancer cells may break away and spread to other areas of the body.


Risk factors

Factors that may increase the risk of angiosarcoma include:

  • Radiation therapy. Treatment with radiation for cancer or other conditions may increase the risk of angiosarcoma. Angiosarcoma is a rare side effect of radiation therapy.
  • Swelling caused by lymph vessel damage. Swelling caused by a backup of lymph fluid is called lymphedema. It happens when the lymphatic system gets blocked or damaged. Lymphedema can happen when lymph nodes are removed during surgery. This is often done during surgery to treat cancer. Lymphedema can also happen when there is an infection or other conditions.
  • Chemicals. Liver angiosarcoma is linked to exposure to several chemicals. Examples of these chemicals include vinyl chloride and arsenic.
  • Genetic syndromes. Certain gene changes that people can be born with can raise the risk of having angiosarcoma. Examples include the gene changes that cause neurofibromatosis, Maffucci syndrome, or Klippel-Trenaunay syndrome, and the BRCA1 and BRCA2 genes.

May 17, 2022

  1. Sturm EC, et al. Multidisciplinary management of angiosarcoma — a review. Journal of Surgical Research. 2020; doi:10.1016/j.jss.2020.07.026.
  2. Bolognia JL, et al., eds. Vascular neoplasms and neoplastic-like proliferations. In: Dermatology. 4th ed. Elsevier; 2018. https://www.clinicalkey.com. Accessed Mar. 13, 2022.
  3. Soft tissue sarcoma. National Comprehensive Cancer Network. https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1464. Accessed Jan. 7, 2022.
  4. Patel SH, et al. Angiosarcoma of the scalp and face: The Mayo Clinic experience. JAMA Otolaryngology — Head & Neck Surgery. 2015;141; doi:10.1001/jamaoto.2014.358.
  5. Goldblum JR, et al. Malignant vascular tumors. In: Enzinger and Weiss's Soft Tissue Tumors. 7th ed. Elsevier; 2020. https://www.clinicalkey.com. Accessed Jan. 7, 2022.
  6. Florou V, et al. Current management of angiosarcoma: Recent advances and lessons from the past. Current Treatment Options in Oncology. 2021; doi:10.1007/s11864-021-00858-9.

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