Overview

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

Angiosarcoma can occur anywhere in your body, but it most often occurs in the skin on your head and neck. Rarely, angiosarcoma may form in the skin on other parts of your body, such as the breast. Or it may form in deeper tissue, such as the liver and the heart. Angiosarcoma can occur in areas previously treated with radiation therapy.

Angiosarcoma treatment depends on where the cancer is located. Treatment options 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, particularly the scalp. Signs and symptoms of this form of angiosarcoma include:

• A raised, purplish 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 surrounding skin

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 doctor if you have any persistent signs or symptoms that worry you.

Causes

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

Doctors know that something happens that causes a cell in the lining of a blood vessel or lymph vessel to develop an error (mutation) in its genetic code. The mutation tells the cell to grow quickly, making more abnormal cells. The abnormal cells continue living when other cells would die.

The result is a buildup of abnormal cells that grows from the affected blood vessel or lymph vessel. With time, cells may break off and spread (metastasize) to other areas of the body.

Risk factors

Factors that may increase your risk of angiosarcoma include:

• Radiation therapy. Treatment with radiation for cancer or other conditions may increase your risk of angiosarcoma. A rare complication of radiation therapy, angiosarcoma typically occurs five to 10 years after treatment.
• Swelling caused by lymph vessel damage (lymphedema). Lymphedema is swelling caused by a backup of lymph fluid that occurs when the lymphatic system is blocked or damaged. Lymphedema is a risk whenever lymph nodes are removed during surgery — a technique that's often used to treat cancer. Lymphedema can also occur in response to infection or other conditions.
• Chemicals. Liver angiosarcoma has been linked to exposure to several chemicals, including vinyl chloride and arsenic.

Diagnosis

Tests and procedures used in angiosarcoma diagnosis include:

• Physical exam. Your doctor will thoroughly examine you to understand your condition.
• Removing a sample of tissue for testing (biopsy). Your doctor will remove a sample of suspicious tissue for laboratory testing. Analysis in the lab can detect cancer cells and determine certain characteristics of your cancer cells that may help guide your treatment.
• Imaging tests. Imaging tests can give your doctor an idea of the extent of your cancer. Tests may include MRI, CT and positron emission tomography (PET). Which tests you undergo will depend on your particular situation.

Treatment

Which angiosarcoma treatment is best for you depends on your cancer's location, its size and whether it has spread to other areas of your body.

Treatment options may include:

• Surgery. The goal of surgery is to remove the angiosarcoma entirely. Your surgeon will remove the cancer and some of the healthy tissue that surrounds it. In some cases surgery may not be an option, for example, if the cancer is very large or has spread to other areas of the body.
• Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells. Radiation therapy is sometimes used after surgery to kill any cancer cells that remain. Radiation therapy may also be an option if you can't undergo surgery.
• Chemotherapy. Chemotherapy is a treatment that uses drugs or chemicals to kill cancer cells. Chemotherapy may be an option if your angiosarcoma has spread to other areas of your body. In certain situations, it may be combined with radiation therapy if you can't undergo surgery.

Preparing for an appointment

If you have signs and symptoms that worry you, start by seeing your family doctor. If your doctor suspects you may have angiosarcoma, you may be referred to a doctor who specializes in skin diseases (dermatologist) or one that specializes in treating cancer (oncologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared. Here's some information to help you get ready, and what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements that you're taking.
• Take a family member or friend along. Sometimes it can be difficult to recall all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For angiosarcoma, some basic questions to ask your doctor include:

• How advanced is my angiosarcoma?
• Has my angiosarcoma spread to other parts of my body?
• What treatments do you recommend?
• What are the benefits and risks of each treatment option?
• I have other health problems. How can I best manage them together?
• Will I be able to work and do my usual activities during angiosarcoma treatment?
• Should I seek a second opinion?
• Should I see a doctor who specializes in cancer treatment?
• How quickly do I need to make a decision about treatment? Can I take some time to consider my options?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?

If any additional questions occur to you during your visit, don't hesitate to ask.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over points you want to talk about in-depth. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Are your symptoms occasional or continuous?
• How severe are your symptoms?
• Does anything seem to improve your symptoms?
• Does anything seem to make your symptoms worse?
• Have you been diagnosed with any other medical conditions?
• What medications are you currently taking, including vitamins and supplements?