Approach

Therapeutic goals include:

  • Palliation of symptoms

  • Prevention of disease progression

  • Cosmetic improvement.

Treatment is individualized according to prognosis and the desired outcome of therapy. Indications for treatment are similar for the different epidemiologic forms of KS (classic [sporadic]; endemic [observed in sub-Saharan Africa]; HIV-associated; iatrogenic [transplant-related]).

Observation may be an option for patients without HIV who have cosmetically acceptable lesions and no other symptoms. People with HIV with limited asymptomatic cutaneous disease can receive antiretroviral therapy (ART) alone.[2]​ For patients with iatrogenic KS, careful reduction of immunosuppressive therapy should be considered. Local and/or systemic therapy can be considered if there is rapid progression, or for patients with symptomatic and/or cosmetically deforming or stigmatizing disease.[2][3][5]​​​​ People with HIV who require treatment should receive the least toxic therapy possible.

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. These patients may require supportive care for nutritional, mobility, social, and psychological wellbeing; to control pain; and to monitor and treat disease complications if they occur.

Local therapy

Considered for individual cutaneous lesions (symptomatic and/or cosmetically disturbing) and for patients unable to tolerate systemic therapy.[5] Options include:[2][3][5]​​​

  • Topical therapies (e.g., retinoids such as alitretinoin, or imiquimod)

  • Radiation therapy

  • Intralesional chemotherapy (vinblastine or bleomycin)

  • Cryotherapy

  • Marginal excision

  • Electrodesiccation and curettage.

Marginal excision, electrodesiccation and curettage, cryotherapy, and intralesional chemotherapy are typically reserved for local control of small, symptomatic lesions. Wide local excision for negative margins is not indicated for KS.[2]​ Cryotherapy should be performed by a clinician with expertise in cutaneous cancer cryotherapy.[2]

Radiation therapy is an effective local treatment; preferred for larger, deeper lesions when systemic therapy is not suitable or effective.[2][3][5]​​ Various radiation therapy dosing regimens have been used. Localized, bulky lesions, particularly those that are ulcerated or infected, may be treated with local external beam radiation therapy.[54]​ Patients should be informed of the risk of secondary cancers, radiation therapy-induced toxicity, and other adverse effects. Lower-dose regimens can reduce toxicity, and are preferred for smaller, superficial lesions, and palliative therapy.[2]

Systemic therapy

Indications for systemic treatment include:[5]

  • Widespread skin involvement

  • Extensive mucosal lesions

  • Symptomatic edema

  • Rapidly progressive disease

  • Symptomatic visceral disease

  • KS flare (exacerbation).

Liposomal doxorubicin is recommended as first-line treatment.[2]​​[3][5]​​​​​​ Myelosuppression is the most important dose-limiting toxicity with liposomal doxorubicin, while neuropathy and alopecia occur infrequently. Cardiotoxicity is very rare, even after the administration of high cumulative doses.

Paclitaxel is an effective second-line treatment. However, there is an increased incidence of alopecia, myalgia, and arthralgia as well as the potential for aggravating preexisting neuropathy.

Nanoparticle albumin-bound (nab)-paclitaxel may be an alternative option if the patient is not able to tolerate paclitaxel.[2][55]​​

ART for HIV-associated KS (formerly known as epidemic or AIDS-related KS)

Initiation of ART is strongly recommended for all HIV-infected patients, regardless of CD4+ T-cell count.[56][57]​​​​​ ART should be started immediately after HIV diagnosis (on the day of diagnosis, if possible, and within 7 days) to increase uptake and engagement, and accelerate the time to viral suppression.[58][59]​​​

Initiation should not be delayed in people with confirmed HIV infection while awaiting testing results, including safety testing and drug-resistance testing; the regimen can be modified when these results are reported.[56]​ See HIV infection.

Optimal control of HIV infection using ART is a key component in the treatment of HIV-associated KS. A stage-stratified approach to managing patients with HIV-associated KS appears to be beneficial; one Cochrane review reported that, in patients with either severe or progressive KS, ART plus chemotherapy may reduce disease progression compared with ART alone.[60]

Patients who are on ART alone should be reassessed within 4 weeks of starting treatment, including monitoring for immune reconstitution inflammatory syndrome (IRIS).[2]​ See Complications.

Immunosuppression for iatrogenic (transplant-related) KS

Consider adjusting immunosuppression for iatrogenic KS. In renal transplant recipients, discontinuation of immunosuppressive therapy is an option if dialysis is available.[20][61]​ Modifying the dose of immunosuppressive drugs may be more difficult in patients with a heart or liver transplant.

Switching to sirolimus for immunosuppression may be sufficient for KS control and treatment; for aggressive or advanced disease, systemic therapy may be given alongside sirolimus.[2][3][5]

Refractory or relapsed KS

Pomalidomide, bortezomib, gemcitabine, lenalidomide, or vinorelbine can be considered as first-line for patients with refractory/relapsed KS.[2][62][63][64][65][66][67][68]​​ Each of these treatment options can be tried in any order, and a specific drug can be repeated (if tolerated and the duration of response is 3 months or more).[2]​ Myelosuppression and thromboembolic complications may occur.

In the US, pomalidomide is approved under accelerated approval for patients with HIV-associated KS whose disease has progressed despite the initiation of ART, or in patients with KS who are HIV-negative.[62][63]​​​​​

Patients taking pomalidomide or lenalidomide must follow strict contraceptive practices as these agents are teratogenic. In the US, patients and medical staff must adhere to a Risk Evaluation and Mitigation Strategy (REMS) to ensure that the benefits of the medication outweigh its risks.

Therapeutic agents that may be useful as further options in the refractory/relapsed setting include:[2]​​[69][70][71][72]​​​[73][74][75]

  • Etoposide

  • Imatinib (a tyrosine kinase inhibitor)

  • Nivolumab (an antiprogrammed death-1 [anti-PD-1] monoclonal antibody immune checkpoint inhibitor) with or without ipilimumab (an anti-cytotoxic T lymphocyte-associated protein-4 [CTLA-4] monoclonal antibody immune checkpoint inhibitor)

  • Pembrolizumab (an anti-PD-1 monoclonal antibody immune checkpoint inhibitor).

Immune checkpoint inhibitors should not be used in patients with concomitant multicentric Castleman disease (MCD) or KS inflammatory cytokine syndrome because of the risk of exacerbation. Consider additional monitoring if using immune checkpoint inhibitors in patients with a history of Kaposi sarcoma-associated herpesvirus (KSHV)-associated diseases.[2]​ See Complications.

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