Kaposi's Sarcoma: Staging and Treatment

How are staging and treatment determined for Kaposi sarcoma?

Staging is the process of learning how much cancer is in your body and where it is. There is no official staging system for KS like there is for many cancers. However, most providers use the AIDS Clinical Trials Group (AGTG) system. The AGTG system focuses more on the strength of your immune system and if you have other AIDS-related infections.

For KS, tests like biopsy, blood tests like a CBC, chest x-ray, CT scan, bronchoscopy, endoscopy, and colonoscopy may be used to help learn more about your cancer. Your providers need to know about your cancer and your health so that they can plan the best treatment for you.

These tests will also help determine what type of KS you have. There are four types:

• AIDS-Associated (“Epidemic”): Most cases of KS in the United States are AIDS-related. People with this type of KS have a severely weakened immune system.
• Mediterranean (“Classic”): Mostly happens in older people from the Mediterranean, Eastern Europe, and the Middle East. Your immune system may be weaker than normal with this type.
• African (“Endemic”): Happens in people living in Equatorial Africa. Many other factors can affect the immune system of people living in this area, such as Malaria and malnutrition.
• Transplant-Related (“Iatrogenic”): When you have a transplant, your immune system will be suppressed (slowed down so your body doesn’t reject the transplant). If you have Kaposi sarcoma--associated herpesvirus (KSHV), your risk of it turning into KS is higher.

Your provider will consider the results of all of your testing and the type of KS you have when determining a treatment plan. They may also look at the ACTG system. The ACTG system looks at three factors:

• The size of your tumor and how far widespread the lesions are.
• How weak your immune system is. This is measured by checking how many immune cells (CD4 cells) are in your blood.
• If you have any other illnesses caused by the cancer or HIV.

The ACTG system for KS also looks at your overall risk factor. This is listed as either 0 (good risk) or 1 (poor risk). The staging system is listed below:

Good risk (all of the following):

T0: Confined to the skin and/or lymph nodes and/or minimal disease in the mouth (non-nodular KS is only on the palate).

I0: CD4+ T-Cell count is greater than 150/μL.

S0: No history of opportunistic infection or thrush. There are no “B” symptoms (fever, night sweats, weight loss, diarrhea for more than 2 weeks). Karnofsky Performance Status is greater than 70 (a measure of how well you can carry out daily activities).

Poor risk (any of the following):

T1: Tumor-associated edema (swelling) or ulcers. There are many lesions in the mouth. There is KS in the GI tract. KS is in organs other than lymph nodes.

I1: CD4+ cell count is less than 150/μL.

S1: History if opportunistic infection and/or thrush. There are “B” symptoms. Karnofsky Performance Status is less than 70. There are other HIV-related illnesses.

How is Kaposi Sarcoma treated?

Treatment for KS depends on many factors, like the type of KS you have, age, immune system status, infections, and testing results. Your treatment may include:

• Treating your weakened immune system and infections.
• Local Therapy.
• Chemotherapy.
• Immunotherapy.
• Clinical Trials.

Treatment of Weakened Immune System and/or Infections

It is important to treat a weak immune system and any infections for AIDS-associated KS and transplant-related KS. If you have HIV or AIDS, medications will be used that treat HIV. These medications are called highly active antiretroviral therapy (HAART). HAART may be the only treatment needed for some patients with AIDS-related KS. If you have transplant related KS, the medications used to slow down your immune system may need to be changed or the dose lowered. Sirolimus and everolimus are medications that prevent your body from rejecting a transplant and can also help with KS.

It is important to do what you can to lower your chance of infection, and to treat active infections as quickly as possible. Talk with your care team about your risk of infection and how to protect your immune system.

Local Therapy

Local therapy focuses on treating the lesions caused by KS. This treatment is often used if there are a few lesions in one spot. Topical therapy only treats the lesion(s) that the therapy is applied to, and it won’t stop new lesions from forming.

• Topical Treatment: Medicine is placed directly onto a lesion. Examples are alitretinoin and Imiquimod.
• Cryosurgery: Liquid nitrogen is used to freeze the lesion and the KS cells are killed. There are no incisions (cuts) of the skin.
• Surgery: If there are only a few, small KS lesions, surgery may be an option to remove them. The lesion can either be cut out (simple excision) or removed with a sharp-edged tool and an electric needle (curettage and electrodesiccation).
• Chemotherapy: Intralesional chemotherapy injects a chemotherapy medication directly into the lesion. Vinblastine may be used.
• Photodynamic Therapy (PDT): A medication is placed on the lesion. Over a few hours or days, the cells in the lesion become more sensitive to certain kinds of light. The lesion is placed under a light source and the cells die.
• Radiation Therapy: The use of high-energy x-rays to kill cancer cells. Radiation is helpful in treating some KS lesions, but sometimes new lesions can appear next to where the radiation was given. Radiation can help with the pain and swelling caused by the lesions.

Chemotherapy

Chemotherapy is the use of anti-cancer medicines that go through your whole body. These medicines may be given through a vein (IV, intravenously) or by mouth. This is unlike when it is used as a local therapy and injected into the lesion. Examples of chemotherapy medications used for systemic treatment of KS are liposomal doxorubicin, liposomal daunorubicin, paclitaxel, nab-paclitaxel, gemcitabine, vinorelbine, bleomycin, vinblastine, vincristine, and etoposide.

It is important to treat a weak immune system and any other infections before starting systemic chemotherapy. Talk with your care team about your treatment plan and if and when chemotherapy may be used.

Immunotherapy

Immunotherapy is the use of medication to help your immune system fight cancer. Examples of immunotherapy medications used for KS are thalidomide, pomalidomide, and lenalidomide.

Clinical Trials

You may be offered a clinical trial as part of your treatment plan. To find out more about current clinical trials, visit the OncoLink Clinical Trials Matching Service.

Making Treatment Decisions

Your care team will make sure you are included in choosing your treatment plan. This can be overwhelming as you may be given a few options to choose from. It feels like an emergency, but you can take a few weeks to meet with different providers and think about your options and what is best for you. This is a personal decision. Friends and family can help you talk through the options and the pros and cons of each, but they cannot make the decision for you. You need to be comfortable with your decision – this will help you move on to the next steps. If you ever have any questions or concerns, be sure to call your team.

You can learn more about Kaposi sarcoma at OncoLink.org.