What Is It?

Kaposi’s sarcoma is a type of cancer that is caused by a virus called human herpes virus 8. The tumors of Kaposi’s sarcoma appear as red or purple patches on the skin, mouth, lungs, liver or gastrointestinal tract. It was considered a rare and relatively benign tumor from the year 1872, when it was first described, until the onset of the AIDS epidemic. The more aggressive form of the disease, which occurs in patients with severely weakened immune systems, is called AIDS-related Kaposi’s sarcoma to distinguish it from the other, more benign forms. With the explosion of HIV/AIDS cases, this form of Kaposi’s sarcoma has become far more common than the other types.

The four types of Kaposi’s sarcoma are:

• Classic Kaposi’s sarcoma — A rare, slow-growing skin tumor, usually affecting males of Italian or Eastern European Jewish ancestry.
• African Kaposi’s sarcoma — Though extremely rare in the rest of the world, Kaposi’s sarcoma occurs more frequently among young males in certain countries in equatorial Africa. Usually it is a slow growing tumor, but in some cases, the skin tumors can be aggressive, invading bone and tissue under the skin.
• Immunosuppressive treatment-related Kaposi’s sarcoma — Patients who are taking immunosuppressive therapy for organ transplantation may develop this form of Kaposi’s sarcoma. It sometimes improves if the immunosuppressive medication is reduced or changed.
• AIDS-related Kaposi’s sarcoma — An opportunistic infection affecting people with late-stage HIV/AIDS. It usually appears as a rapidly progressing tumor affecting the skin, lymph nodes, gastrointestinal tract, lungs, liver and spleen. In the United States, 95 percent of the cases involve homosexual or bisexual men. The incidence of this tumor is decreasing with the advent of highly active retroviral therapy for HIV/AIDS.

The first symptoms of Kaposi’s sarcoma are usually red, purple or brown patches, plaques, or nodules on the skin, often with a bruise-like appearance. In the classic, African and immunosuppressive forms of Kaposi’s sarcoma, these lesions usually are slow-growing, progressing over years. As the disease gets worse, the lower legs may swell and, in some cases, the disease may spread to other organs.

In the AIDS-related form, however, the tumor is much more aggressive, often growing to cover larger areas, and forming tumorlike masses. At first, these lesions are typically soft and spongy, but over time, they usually become hard and solid. The surface of the tumor may form open ulcers, which may become infected. The disease rarely is confined to the skin, and often affects the mouth, lymph nodes, lungs, liver, spleen and gastrointestinal tract. When the tumor involves the lungs, it often causes cough, shortness of breath and wheezing. In the lungs, the disease often progresses rapidly, and may result in death from respiratory failure. However, when the disease involves the gastrointestinal tract, it rarely causes symptoms unless the disease is very advanced. In that case, patients may develop symptoms of intestinal obstruction (nausea, vomiting and abdominal pain) or bloody stools. If the lymph nodes become involved, severe swelling can occur, usually of the legs or face.

Kaposi’s sarcoma usually is suspected based on the appearance of the skin lesions, and it can be confirmed with a biopsy.

If your doctor suspects Kaposi’s sarcoma, he or she will ask you if you are HIV positive or if you have another disease which may be suppressing your immune system. If you do not know if you are infected with HIV, but have HIV risk factors, your doctor will recommend an HIV test.

If you are HIV positive, your doctor will ask you many questions about your experience with this disease:

• When were you diagnosed?
• What medications have you taken, or are you currently taking?
• What other illnesses have you had that are related to the HIV virus (opportunistic infections)?
• What are the results of your most recent lab tests (such as CD4 count and viral load)?

If you have Kaposi’s sarcoma, your doctor will try to establish the extent of spread by asking you several questions and by performing a careful physical examination.

• Are you coughing or short of breath? This could indicate lung involvement.
• Are your legs swelling? This suggests lymph-node involvement.
• Do you have nausea, vomiting, abdominal pain or blood in your stool? This suggests gastrointestinal involvement.

There is no cure for Kaposi’s sarcoma. It is a lifelong condition. However, the symptoms will improve with treatment and with HAART (highly active antiretroviral therapy) for patients with HIV/AIDS.

There is no way to prevent the milder forms of Kaposi’s sarcoma (classic, African, and immunosuppression-related). The most effective way to avoid developing Kaposi’s sarcoma is to prevent the transmission of HIV. HIV-positive patients can decrease their risk of Kaposi’s sarcoma by taking HAART (highly active antiretroviral therapy).

The goal of treatment is to alleviate symptoms, shrink the tumor and prevent the disease from progressing. The type of treatment recommended depends on the type of Kaposi’s sarcoma, the size of the tumor, the extent of internal-organ involvement and the patient’s CD4 count and general medical condition. The four treatment types include:

• Surgery — Removing the cancer cells
• Cryotherapy — Freezing the cancer cells to kill them
• chemotherapy — Using drugs to kill the cancer cells
• Radiation therapy — Using radiation to kill the cancer cells
• Biological therapy — Using the body’s immune system to fight the cancer cells

In patients with AIDS, the lesions will improve with treatment of the AIDS itself. Typically, this involves the use of antiretroviral medications that are prescribed to improve the functioning of the immune system.

See a doctor if you have skin changes that fit the description of Kaposi’s sarcoma. If you are HIV-positive and have Kaposi’s sarcoma, you will need immediate treatment, and your doctor may need to change or add medications to treat the HIV.

If you have not been tested for HIV, and you think you may have Kaposi’s sarcoma, it’s important to talk to your doctor right away. He or she will counsel you about risk factors for HIV and ask if you have symptoms suggestive of HIV/AIDS, such as swollen lymph nodes, night sweats, fevers, fatigue and weight loss. If you are at risk of HIV, a blood test for the AIDS virus may be recommended. Early diagnosis is essential in HIV disease, because the prognosis improves dramatically with treatment.

The prognosis for Kaposi’s sarcoma depends on the form of the disease. The milder forms of Kaposi’s sarcoma are rare and progress slowly. Patients with classic Kaposi’s sarcoma usually die of other causes or develop a second type of cancer, which occurs in up to one-third of people with classic Kaposi’s sarcoma. Immunosuppression-related Kaposi’s sarcoma often will improve with a decrease or change in drug doses.

The most important factor in the prognosis for AIDS-related Kaposi’s sarcoma is the level of immune-system impairment. The development of Kaposi’s sarcoma suggests that the immune system is highly impaired, and patients with this disease usually die of a different HIV-related opportunistic infection. Patients who are taking HAART (highly active antiretroviral therapy) have a better prognosis.

Diseases and Conditions Center

All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.