What is the treatment for Kaposi sarcoma?

From the Guidelines

The treatment for Kaposi sarcoma depends on its extent, location, and the patient's immune status, with antiretroviral therapy (ART) being crucial for HIV-associated Kaposi sarcoma, and first-line chemotherapy often including liposomal doxorubicin or paclitaxel. For localized lesions, local treatments such as cryotherapy, radiation therapy, or surgical excision may be sufficient 1. Topical retinoids like alitretinoin gel can be applied to cutaneous lesions. For more extensive disease, systemic therapy is typically required.

Key Considerations

• The choice of therapy is guided by the clinical variant (classic, endemic, iatrogenic, or AIDS-related), as Kaposi sarcoma is caused by human herpesvirus 8 (HHV-8) infection, with immunosuppression playing a key role in disease development 1.
• For patients with limited cutaneous disease that is asymptomatic and cosmetically acceptable, ART alone may be sufficient, while those with symptomatic and/or cosmetically unacceptable limited cutaneous disease should be treated with ART and minimally invasive therapy with the least toxicity possible 1.
• Preferred initial treatment for patients with advanced cutaneous, oral, visceral, or nodal AIDS-related Kaposi sarcoma is ART with clinical trial or systemic therapy, with liposomal doxorubicin being the preferred first-line systemic therapy 1.
• For relapsed/refractory disease, a typical systemic therapy sequence would be first-line liposomal doxorubicin, followed by second-line paclitaxel, followed by pomalidomide in the third line of treatment 1.
• Radiation therapy can be used for patients with limited cutaneous disease that is symptomatic and/or cosmetically unacceptable, or for palliative therapy to mitigate pain or other symptoms in advanced disease 1.

Treatment Options

• Liposomal doxorubicin (20-40 mg/m² every 2-3 weeks) or paclitaxel (100 mg/m² weekly for 2 weeks with 1 week off) are common first-line chemotherapy options.
• Pomalidomide is recommended as the preferred regimen for third-line therapy.
• Other treatment options for subsequent lines of therapy include bevacizumab, etoposide, gemcitabine, imatinib, interferon, nab-paclitaxel, thalidomide, and vinorelbine, but data for these agents are generally limited.

Important Considerations

• Glucocorticoids should be avoided in patients with active or prior Kaposi sarcoma due to the potential to cause significant flares or relapses 1.
• Regular follow-up is essential to monitor treatment response and detect recurrence.
• The goals of therapy for patients with advanced disease are reducing or reversing symptoms and mitigating end-organ damage, with complete remissions being rare but effective therapy leading to long-term disease control 1.

From the FDA Drug Label

Paclitaxel is indicated for the second-line treatment of AIDS-related Kaposi’s sarcoma. The efficacy of paclitaxel was evaluated by assessing cutaneous tumor response according to the amended ACTG criteria and by seeking evidence of clinical benefit in patients in 6 domains of symptoms and/or conditions that are commonly related to AIDS-related Kaposi’s sarcoma The objective response rate was 59% (95% CI, 46 to 72%) (35 of 59 patients) in patients with prior systemic therapy. The treatment for Kaposi sarcoma is paclitaxel as a second-line therapy, with an objective response rate of 59% in patients with prior systemic therapy 2.

• Key points:
  • Paclitaxel is used for the second-line treatment of AIDS-related Kaposi’s sarcoma.
  • The dose intensity of paclitaxel used in this patient population was lower than the dose intensity recommended for other solid tumors.
  • Efficacy was evaluated by assessing cutaneous tumor response according to the amended ACTG criteria 2.

From the Research

Treatment Options for Kaposi Sarcoma

The treatment for Kaposi sarcoma depends on the severity and extent of the disease. Some of the treatment options include:

• Highly active antiretroviral therapy (HAART) alone for early stage cutaneous AIDS-KS 3
• Systemic chemotherapy in addition to antiretrovirals for advanced stage Kaposi's sarcoma with visceral disease, tumor-associated edema or extensive oral disease 3
• Liposomal anthracyclines as the standard first-line therapy for advanced stage Kaposi's sarcoma, with response rates of around 70% 3
• Paclitaxel for patients with refractory or recurrent AIDS-KS 3
• Local therapeutic options such as excision, cryosurgery, radiation and alitretinoin gel 4
• Interferon-alpha for patients with more than 400/microl CD4+ lymphocytes, which can induce complete remission in 45% of patients 4

Chemotherapy Regimens

Different chemotherapy regimens have been studied for the treatment of Kaposi sarcoma, including:

• Doxorubicin, bleomycin and vincristine (ABV) 5
• Liposomal anthracyclines 3, 5
• Paclitaxel 3, 5
• Pegylated liposomal doxorubicin 5
• Liposomal daunorubicin 5
• Oral etoposide 5
• Bleomycin alone or in combination with vinblastine, vincristine or single-agent antiretroviral therapy alone 5

Efficacy of Treatments

The efficacy of these treatments varies, with some studies showing significant reductions in disease progression and mortality, while others show no difference between treatment regimens 6, 5. The quality of evidence is generally moderate, with many studies having small sample sizes and limited follow-up 5. Further research is needed to determine the most effective treatment options for Kaposi sarcoma.