What are the treatment options for Kaposi sarcoma?

Treatment Options for Kaposi Sarcoma

Treatment of Kaposi sarcoma should be tailored based on disease extent, with antiretroviral therapy (ART) forming the foundation for AIDS-related cases, supplemented by local or systemic therapies depending on disease severity and distribution. 1

Initial Assessment and Classification

• Disease extent should be categorized as either limited cutaneous disease or advanced disease (extensive cutaneous, oral, visceral, or nodal involvement) 1
• Individual Kaposi sarcoma lesions often represent distinct clones rather than metastases, arising from common risk factors of immunosuppression and persistent HHV-8 infection 1
• Persistence of HHV-8 infection results in ongoing risk of recurrence/disease progression, making disease control rather than cure the primary goal 1

Treatment Approach Based on Disease Extent

Limited Cutaneous Disease

• Asymptomatic and cosmetically acceptable disease:

  • ART alone is the preferred initial approach, as remissions or stable disease may occur with optimization of immune function and HIV viral suppression 1

• Symptomatic and/or cosmetically unacceptable disease:

  • ART plus minimally invasive therapy with the least toxicity possible 1
  • Options include:
    • Topical treatments (alitretinoin gel, imiquimod) 1
    • Intralesional chemotherapy 1
    • Radiation therapy 1
    • Local excision 1
    • Limited cycles (3-6) of systemic therapy for those initiating or reinitiating ART 1

Advanced Disease (Extensive Cutaneous, Oral, Visceral, or Nodal)

• Preferred initial treatment:

  • ART plus systemic therapy or clinical trial 1
  • For those not eligible for clinical trial or systemic therapy, radiation can be used with ART 1

• Systemic therapy options:

  • First-line: Liposomal doxorubicin 1
  • Second-line: Paclitaxel (FDA-approved for second-line treatment of AIDS-related Kaposi sarcoma) 2
  • Third-line: Pomalidomide 1
  • Additional options: Interferon alpha-2b, nab-paclitaxel, thalidomide, vinorelbine 1

Special Considerations

• Antiretroviral Therapy (ART):

  • Comanagement with an HIV specialist is crucial to optimize HIV suppression and immune reconstitution 1
  • ART may lead to immune reconstitution inflammatory syndrome (IRIS) in 6-39% of patients within 3-6 months 1
  • IRIS presents with marked lesional swelling, increased tenderness, and peripheral edema 1

• Important Cautions:

  • Glucocorticoids should be avoided in patients with active or prior Kaposi sarcoma due to potential for significant flares or relapses 1
  • Intralesional chemotherapy and radiation to plantar and palmar surfaces should be approached with caution 1
  • Other therapies associated with Kaposi sarcoma flares include those suppressing B- and T-cell function (rituximab, cyclosporine) 1

Follow-up and Disease Monitoring

• If disease progresses on initial therapy, a different treatment option should be tried based on disease extent 1
• If disease is stable or responds to initial therapy, continue ART while monitoring the patient 1
• For relapsed/refractory disease after an initial response, the previously effective therapy may be repeated if the response was durable 1
• Surveillance is important as disease can recur even after complete response and with normal T-cell counts 1

Treatment Outcomes

• The survival of patients with AIDS-related Kaposi sarcoma has greatly improved with modern therapies 1
• Complete remissions in advanced disease are rare, but effective therapy can lead to long-term disease control 1
• Studies show that HAART plus chemotherapy may be more beneficial in reducing disease progression compared to HAART alone in patients with severe or progressive Kaposi sarcoma 3

Emerging Therapies

• Immunomodulatory agents, antiangiogenic therapies, and checkpoint inhibitors are being explored as promising treatment options 4
• For patients with preserved immune systems who are antiretroviral-naive, ART may efficiently reduce progression of Kaposi sarcoma lesions, potentially avoiding chemotherapy 5