Kaposi sarcoma is a rare type of cancer accounting for less than 1% of all cancer diagnoses. If you or a loved one have been diagnosed with Kaposi sarcoma, our experts will provide information and treatment options to help you navigate your diagnosis. The OSUCCC – James is home to central Ohio’s only Sarcoma Clinic and specializes in seeing and treating this type of cancer. Our dedicated, full-time sarcoma oncologists are specially trained to diagnose and treat Kaposi sarcoma and provide access to some of the latest research and treatment offerings.
What is Kaposi sarcoma?
Kaposi sarcoma is a rare type of sarcoma that occurs specifically in the body’s soft tissues. Soft tissues are any tissues that surround or support other structures within the body, including connective tissue such as nerves, blood vessels or tendons. Therefore, Kaposi sarcoma primarily affects blood vessels. This cancer causes purple, red or brown lesions to form in the skin, mucous membranes, lymph nodes and organs. The lesions get their color from the lining of the blood vessels and can appear on different parts of the body and even at the same time.
What causes Kaposi sarcoma?
The primary cause of this cancer type is a virus called human herpes virus-8 (HHV-8) or Kaposi sarcoma virus. HHV-8 is in the same family of viruses as the Epstein-Barr virus, which causes infectious mononucleosis (mono).
Our cells contain instructions, called DNA, to help them grow and multiply. If a cell’s DNA changes over time, this can cause the cell to generate “bad instructions” when it reproduces. When this happens, a cell forms abnormally and eventually may become cancerous.
In the case of Kaposi sarcoma, the presence of HHV-8 in the body can influence changes in a cell’s growth and reproduction instructions. As a result, cancer cells may form in the soft tissues. If this occurs, HHV-8 influences how the abnormal cells behave in the body when compared to healthy normal cells.
Most who have HHV-8 never develop Kaposi sarcoma, but those who carry the virus and who suffer from a weakened immune system are at a higher risk for developing it.
Kaposi sarcoma symptoms
Symptoms for Kaposi sarcoma may vary. If you are experiencing any symptoms below, talk to your doctor.
Main symptoms include:
- Purple, red or brown marks (lesions) on the skin or within mucous membranes lining the mouth, nose and throat
- Lesions may look like a bruise Lesions may also appear in lymph nodes or organs
Additional symptoms may include:
- Difficulty swallowing or speaking
- Unexplained cough or breathing issues
- Pain when walking
- Unexplained swelling near the lymph nodes
- Abdominal pain and diarrhea
- Unexplained fatigue
If you notice any unusual symptoms, it’s important to see your doctor so that they can properly exam and diagnose you.
Types of Kaposi sarcoma
Like all cancers, there are different types of Kaposi sarcoma. Your specific type will determine possible treatment approaches and more. At the OSUCCC – James, our team of sarcoma experts will properly diagnose and treat your specific type of Kaposi sarcoma to ensure the best possible treatment outcomes.
The main types of Kaposi sarcoma include:
Classic Kaposi sarcoma
If you have classic Kaposi sarcoma, you may develop dark-colored lesions, often on the legs, ankles or feet. These lesions are slow-growing and don’t often cause any symptoms. Over time, however, they can cause problems such as painful leg swelling or gastrointestinal bleeding.
Males assigned at birth of Italian or Eastern European descent are more susceptible to this form of the disease, and if you have classic Kaposi sarcoma, you can often develop a secondary cancer, which is most often non-Hodgkin’s lymphoma.
African Kaposi sarcoma
This disease is found most frequently in young adult males assigned at birth who live near the equator in Africa. It follows a similar course to classic Kaposi sarcoma and is characterized by violet to dark brown skin lesions. A more aggressive form can spread from the skin to other tissues or organs.
Immunosuppressive therapy-related Kaposi sarcoma
Also called transplant-related or acquired Kaposi sarcoma, this form of sarcoma can occur after a major organ transplant such as a kidney, heart or liver transplant. Those who undergo these types of major surgeries often take drugs to suppress the immune system, and Kaposi sarcoma may develop because of the weakened immune systems. This form of the disease often affects the skin but can spread to other parts of the body, including mucous membranes or other organs.
Epidemic Kaposi sarcoma
Epidemic Kaposi sarcoma is seen only in homosexual and bisexual males assigned at birth who are infected with human immunodeficiency virus (HIV). If you have HIV and it develops into acquired immunodeficiency syndrome (AIDS), you will have a weakened immune system and are much more susceptible to infections and cancers such as Kaposi sarcoma. You may benefit from a combination of antiviral drugs called highly active antiretroviral therapy. This treatment may slow down the development of AIDS and AIDS-related Kaposi sarcoma.
Over time, the disease may spread to other parts of the body and cause more severe symptoms such as fever, weight loss and diarrhea.
Non-epidemic Kaposi sarcoma
Non-epidemic Kaposi sarcoma most often develops in homosexual and bisexual males assigned at birth who have no signs or symptoms of HIV infection. This is a much slower-growing cancer, it is extremely rare, and lesions and related symptoms are treatable in most cases.
Kaposi sarcoma prevention
At the OSUCCC – James, our cancer research experts focus on all aspects of studying Kaposi sarcoma — from prevention to treatment. Home to one of the top sarcoma research programs in the country, we are working hard to understand rare cancers like Kaposi sarcoma.
There is no way to prevent Kaposi sarcoma; however, there are some risk factors you can influence and others you can’t. Risk factors are anything that may increase your likelihood of developing a condition. It’s important to remember that just because you have a risk factor doesn’t necessarily mean you’ll get Kaposi sarcoma.
Some risk factors for Kaposi sarcoma include:
- Prior infection with Kaposi sarcoma-associated herpesvirus (KSHV) or human herpesvirus 8 (HHV-8)
- Exposure to HIV/AIDS: If you have been exposed to HIV/AIDS through unprotected sex or have shared needles with others
- Not taking medications to control an active HIV infection
- Not taking specific immune suppressive drugs after organ transplants
Kaposi sarcoma screening and diagnosis
If you are at high risk for developing Kaposi sarcoma, specialists at the OSUCCC – James can determine if you need specific testing or further evaluation. Some of these tests may include imaging, biopsies or a combination of both.
The following tests can help detect and diagnose Kaposi sarcoma:
Kaposi sarcoma biopsies
A pathologist will remove a small piece of tissue to analyze under a microscope for signs of cancer. There are several ways to obtain a biopsy sample for diagnosing Kaposi sarcoma including:
- Incisional biopsy
- Core biopsy
- Excisional biopsy
Imaging tests
There are some imaging tests that can also be ordered to screen for Kaposi sarcoma, which may include:
- Chest X-ray
- Endoscopy
- Bronchoscopy
The type of tests ordered will be dependent on several factors, and your OSUCCC – James specialist will work with you to determine the right combination of tests for your needs.
Kaposi sarcoma treatment
If tests determine that you have Kaposi sarcoma, your doctor may recommend one or a combination of different treatment types.
One or more of the following treatment therapies may be recommended:
Surgery
Most patients who have Kaposi sarcoma have surgery. Once the cancer is surgically removed, patients may undergo additional radiation or chemotherapy after surgery to kill any cancer cells that may not have been able to be retrieved during surgery. This lowers the risk of the cancer coming back. You may be eligible for surgery to treat surface lesions, particularly if they are small.
Surgery may consist of one of the following procedures:
- Local excision: An OSUCCC – James surgeon removes the lesion and some normal tissue around the tumor. The surgery also is called wide local excision.
- Electrodesiccation and curettage: A surgeon removes the cancerous lesion using an instrument called a curette. An electric current is used to stop bleeding and destroy any remaining cancer cells.
- Cryosurgery or cryotherapy: This treatment freezes the cancerous lesion and destroys it with small doses of liquid nitrogen.
Radiation therapy
Radiation therapy uses high-energy X-rays or other radiation to kill cancer cells or keep them from growing. You may receive radiation therapy from a radiation oncologist before or after a surgery that removes a tumor. Radiation can shrink a tumor to make surgery more successful. Postoperative radiation therapy is used to destroy any remaining cancer cells.
Chemotherapy
Chemotherapy drugs stop cancer cell growth by either destroying the cells or stopping them from dividing. These medications can be taken by mouth or injected into a vein or muscle, and they may be given over a period of months.
Localized Kaposi sarcoma lesions may be treated with a chemotherapy drug that is injected directly into the lesion or the drug may be applied topically as a gel. Chemotherapy may also be delivered to lesions by liposomes (microscopic fat particles) carrying a drug such as doxorubicin.
When the drugs enter the bloodstream, they can reach cancer cells throughout the body (called systemic chemotherapy). Combination chemotherapy uses more than one anti-cancer drug.
Immunotherapy for Kaposi sarcoma
Immunotherapy, also called biologic therapy, is a treatment that uses your immune system to fight cancer. Substances made by your body, or made in a laboratory, can be used to encourage your body’s immune system to activate and attack Kaposi sarcoma cells in the body.
One type of immunotherapeutic treatment used to treat Kaposi sarcoma is interferon alfa.
Targeted therapy
Targeted therapy is a type of drug treatment designed to target and attack cancer cells, leaving healthy or normal cells unharmed. These drugs often have less severe side effects and are generally better tolerated than chemotherapy drugs.
If you’ve been diagnosed with Kaposi sarcoma, would like a second opinion or would like to speak with a sarcoma specialist, please call The James Line at 800-293-5066 or 614-293-5066 to make an appointment.
Additional Resources
Types of Sarcoma
Soft Tissue Sarcoma
Head and Neck Sarcoma
Screening and Diagnosis
Clinical Trials