Kaposi Sarcoma: Risk Stratification and Treatment

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No universally accepted staging system for Kaposi Sarcoma is available. The AIDS Clinical Trials Group (ACTG) described risk group stratification system for Epidemic/AIDS-related KS is the most widely used grading system. This system divides Epidemic/AIDS-related KS into 2 risk groups (i.e. Good and Poor) based on the extent of disease, status of the immune system, and presence of systemic illness.

Following table describes the characteristics of the 2 risk groups:

Good (Low) RiskPoor (High) Risk
Localized disease
CD4 cell count >/=150 cells/mm^3
Widespread disease
CD4 cell count <150 cells/mm^3
No systemic illness present (No opportunistic infections, No B symptoms, Karnofsky performance status score >/=70)Systemic illness present (History of opportunistic infection, B symptoms, Karnofsky performance status score <70, or HIV-related illness, such as neurological disease or lymphoma)

The Kaposi sarcoma treatment usually depends on many factors, including but not limited to the type of sarcoma, location of the disease, patient’s age and performance status, patient’s preference, along with other factors. Following are the preferred treatment approaches for different types of Kaposi sarcoma, but the final decision is taken after clinical assessment of the patient by an oncologist.

TYPETREATMENT
Epidemic/ AIDS-related KSFor patients with AIDS-related KS, treatment of the HIV infection with highly active antiretroviral therapy (HAART) is considered the primary treatment. Treatment of AIDS leads to better immune function and relieves the symptoms of KS as well. Prophylactic treatment for opportunistic infection or immediate treatment of any infection should be provided as supportive care.

For patients with localized disease (good risk patients) and presence of symptoms, besides HAART, a local treatment is usually employed, which may include radiation therapy, cryosurgery, a topical retinoid, intralesional chemotherapy, or local excision.

For patients with widespread disease (patients with poor risk), lymphedema, or other serious complications, HAART and systemic chemotherapy are usually employed to control disease symptoms. Targeted drugs or immunotherapeutic agents may be employed alone or in combination with chemotherapy to slow down or delay the progression of the disease. HAART should be continued with the cessation of chemotherapy and/or targeted therapy upon resolution of skin lesions.
Classic/ Mediterranean KSClassic KS grow and spread very slowly and do not cause any symptom unless it has spread to vital body parts. These patients are also at a high risk of developing secondary cancer and should be followed up closely to monitor the same.

For patients with localized disease, surgery, radiotherapy, or intralesional chemotherapy is considered as the preferred treatment.

For patients with disseminated disease, chemotherapy is usually employed to control disease symptoms. Radiotherapy may be added to chemotherapy for local/superficial disease control.
Endemic/ African KSEndemic Ks is usually observed in poor countries where treatment options are limited. When possible treatment similar to that of classic KS should be employed for endemic KS.
Iatrogenic/ transplant-related KSFor patients with transplant-related KS, cessation or dose reduction of the immunosuppressive agents is recommended to ease the disease symptoms. In place of conventional immunosuppressive drugs, angiogenesis inhibitor (e.g. sirolimus) may be employed as anti-rejection treatment. This enables the immune system to recover and fight against the KS cells. Radiotherapy and other local treatments can be employed to relieve the local disease symptoms.

Local Therapy for Kaposi Sarcoma: Patients with Kaposi sarcoma (KS) generally required local therapy for controlling localized/superficial disease lesions. Topical agents, for example, alitretinoin, a retinoid drug may be used topically to relieve pain and swelling associated with these lesions. Local excision may be employed using simple surgical excision, curettage (tumor removal by scraping), electrodesiccation (tumor cell destruction using electric current), or cryosurgery (killing cancer cells by intense cooling). Intralesional chemotherapy (injecting the chemotherapeutic drug directly into the KS lesion) can also be used for local disease control. These treatments help in controlling local disease symptoms and do not cause systemic side-effects.

Palliative Treatment: It do not directly treat KS but helps in improving the overall quality of life by providing relief from the symptoms and by reducing the suffering caused by the disease. It is generally given as supportive care for advanced staged KS along with other treatments. It may include but not limited to: using drugs or other interventions to reduce swelling, pain, bleeding, and other symptoms such as nausea, vomiting, and diarrhea; surgical interventions or radiotherapy may also be employed.

It is very important to assess the benefits of each treatment option versus the possible risks and side effects before making a treatment decision. Sometimes, patient’s choice and health condition are also important to make a treatment choice.

Following are the goals for Kaposi sarcoma treatment:

  • Prolongation of life.
  • Reduction of symptoms.
  • Improvement of overall quality of life.
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