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What

Angiosarcoma, also called haemangiosarcoma, is an extremely rare form of cancer. It is known as a malignant neoplasm. malignant means it can spread and invade other tissues; neoplasm, another word for tumor, means “new growth.” Angiosarcomas are created by a change in endothelial cells, the cells that normally make up blood vessels, such as arteries and veins. Angiosarcomas belong to the class of cancers known as sarcomas, which spread through the blood system and infiltrate other parts of the body. Angiosarcomas are aggressive cancers. They proliferate rapidly, and cancer cells from the main tumor spread widely through the body. When tumors are removed, they tend to arise again (recur) locally. It is a difficult disease to treat and cure. Angiosarcomas are rare. In fact, physicians in the United States diagnose only about 60 new cases of soft tissue angiosarcoma each year, plus about 25 cases of angiosarcoma in the liver and eight in the kidney. Angiosarcomas can occur in any region of the body, including the skin, soft tissues, liver, breast, spleen, bone, heart and muscles. They can arise in the extremities, the head and neck, the abdominal wall and retroperitoneum (the tissue at the back of the abdominal cavity).

Who

In general, angiosarcoma most commonly affects older people, but children can be affected.

Signs and Symptoms

ecause angiosarcomas can grow in so many kinds of tissue, symptoms can vary widely. Angiosarcoma of the soft tissue (extremities, retroperitoneum, abdominal wall) may appear as a growing mass. In bone, it may appear as a tumor of the skeleton, long bones, hands or feet. On the skin, it can appear as an enlarging bruise, a blue-black nodule or an unhealed ulceration. These can be confused with other problems, such as cellulitis, edema, bruising or infection, which may delay the correct diagnosis. Skin lesions may be painful and bleed. On the head and neck, angiosarcoma can show up as ulcerated nodules, plaques or bruises. Sometimes, the tumors are accompanied by swelling. Patients with liver angiosarcoma may have nonspecific symptoms, such as fatigue, weight loss and pain in the upper right part of their bodies . The skin may turn yellow (jaundice). In the lungs, initial symptoms include chest pain, weight loss, cough and labored breathing. However, some patients show no symptoms at all. Angiosarcoma in the heart can go unnoticed for a long time, or it can be mistaken for other heart ailments, such as inflammation or restricted blood flow.

Possible Causes

Angiosarcoma has been linked to contact with toxic substances and radiation, including radiotherapy. People exposed to the toxic metal arsenic, polyvinyl chloride (PVC used in plastics), and Thorotrast® (a radioactive contrast dye once used in radiology) may be more likely to develop this cancer. Some evidence shows that foreign material introduced into the body, such as shrapnel, implanted metal devices, plastic grafts, steel and some synthetic, may be associated with angiosarcoma. Certain steroids used to treat other medical conditions may also be linked, along with exposure to anti-mildew solutions in agriculture, and arsenic-containing Fowler solution in the management of psoriasis.

Diagnosis

A biopsy will be needed to determine whether or not your child has angiosarcoma. If the diagnosis is angiosarcoma, the doctor will determine what stage the tumor has reached, which will confirm whether or not the tumor has spread from its original location, and, if so, how far. “Staging” helps doctors decide what kind of treatment is best, and gives an idea as to what the prognosis will be. This is accomplished through noninvasive imaging studies that may be needed, including X-rays, Magnetic Resonance Imaging (MRI), a Computerized Tomography scan (CT or CAT scan) and bone scans. Blood tests probably will be ordered, as well.

Treatment

Surgery to remove the affected tissue will likely be the preferred treatment. Depending on where the problem is, radiation therapy may be used by itself or in combination with surgery. Chemotherapy may also be used in the course of your child’s treatment. New drugs, such as anti-angiogenesis agents (which stop the growth of new blood vessels), may open the door to treatment of this rapidly fatal disease.

Prognosis

In children, angiosarcoma tends to be somewhat less serious than in adults, and children can have a better prognosis. However, angiosarcoma is a very difficult disease to treat and can be lethal. Angiosarcoma tends to recur locally, as well as spread to the lung and lymph nodes. The earlier it is diagnosed, the better.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

E-Medicine
An extensive review article written for healthcare professionals.  A website with detailed information about the disease and treatment options.

The Sarcoma Alliance
A general website with information on all types of sarcomas, including some specific to AMFH, that provides education, guidance and support for parents and families affected by the disease.

University of Alabama Health Sciences
A website with lists of resources for patients and families affected by Angiosarcoma.

National cancer Institute
A good website for general information, links to current research and support groups. There are also nice definitions for many technical terms.

Google Search for Angiosarcoma

References and Sources

www.emedicine.com/med/topic138.htm www.sarcomaalliance.com/links.html www.cchs-dl.slis.ua.edu/petientinfo/oncology/sarcomas/soft-tissue/angiosarcoma.html www.cancer.gov/cancerinfo/types