Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

HIV-associated KS

1st line – antiretroviral therapy (ART)

Back
ACUTE
|
HIV-associated KS
1st line – 

antiretroviral therapy (ART)

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 test results, including safety testing and drug-resistance testing; the regimen can be modified when these results are reported.[56] 

People with HIV with limited asymptomatic cutaneous disease can receive ART alone.[2]​ 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.

HIV-treatment prescribing is best individualized by an experienced HIV clinician. See HIV infection.

Consider – local therapy

Back
ACUTE
|
HIV-associated KS
|
with localized lesion(s)
Consider – 

local therapy

Treatment recommended for SOME patients in selected patient group

Considered for individual cutaneous lesions (symptomatic and/or cosmetically disturbing) and for patients unable to tolerate systemic therapy.[5]​ Options include: topical therapies (e.g., retinoids such as alitretinoin, or imiquimod); radiation therapy; intralesional chemotherapy (vinblastine or bleomycin); cryotherapy; marginal excision; and electrodesiccation and curettage.[2][3][5]

Alitretinoin is self-administered and patients usually require at least 4-8 weeks of treatment. Imiquimod is also self-administered. It requires at least 24 weeks of treatment, and is usually applied under occlusion for 10-12 hours at a time. Dermal irritation at the site of application is common.

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.[2] 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]

People with HIV who require treatment should receive the least toxic therapy possible.

Primary options

alitretinoin topical: (0.1%) apply to the affected area(s) twice to four times daily

OR

imiquimod topical: (5%) apply to the affected area(s) three times weekly initially, titrate frequency of application to tolerance and effect up to once daily

OR

vinblastine: consult specialist for guidance on intralesional dose

OR

bleomycin: consult specialist for guidance on intralesional dose

Plus – systemic therapy

Back
ACUTE
|
HIV-associated KS
|
with widespread lesions ± systemic involvement
Plus – 

systemic therapy

Treatment recommended for ALL patients in selected patient group

Indications for systemic treatment include: widespread skin involvement; extensive mucosal lesions; symptomatic edema; rapidly progressive disease; symptomatic visceral disease; and KS flare (exacerbation).[5]

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. Acute infusion reactions are characterized by back pain, shortness of breath, and intense flushing. These reactions typically occur within moments of starting the infusion and subside rapidly after drug discontinuation. Infusions are sometimes able to be resumed at a slower rate.

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. Reduced corticosteroid premedication should be used and people with HIV should be monitored for toxicity related to possible antiretroviral drug interaction.

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

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, antiretroviral therapy (ART) plus chemotherapy may reduce disease progression compared with ART alone.[60]

People with HIV who require treatment should receive the least toxic therapy possible.

See local specialist protocol for dosing guidelines.

Primary options

doxorubicin liposomal

Secondary options

paclitaxel

Tertiary options

paclitaxel nanoparticle albumin-bound

Plus – supportive care

Back
ACUTE
|
HIV-associated KS
|
with widespread lesions ± systemic involvement
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

Plus – systemic therapy

Back
ACUTE
|
HIV-associated KS
|
with refractory or relapsed KS
Plus – 

systemic therapy

Treatment recommended for ALL patients in selected patient group

Pomalidomide, bortezomib, gemcitabine, lenalidomide, or vinorelbine can be considered as first-line options 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 antiretroviral therapy (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 for KS in the refractory/relapsed setting include etoposide; imatinib (a tyrosine kinase inhibitor); nivolumab (an anti-programmed death-1 [anti-PD-1] monoclonal antibody immune checkpoint inhibitor) with or without ipilimumab (an anticytotoxic T lymphocyte-associated protein-4 [CTLA-4] monoclonal antibody immune checkpoint inhibitor); pembrolizumab (an anti-PD-1 monoclonal antibody immune checkpoint inhibitor).[2][69][71][75]​​​​​

Immune checkpoint inhibitors should not be used in patients with concomitant multicentric Castleman disease (MCD) or KS inflammatory cytokine syndrome (KICS) 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.

Primary options

pomalidomide

OR

bortezomib

OR

gemcitabine

OR

lenalidomide

OR

vinorelbine

Secondary options

etoposide

OR

imatinib

OR

nivolumab

OR

nivolumab

and

ipilimumab

OR

pembrolizumab

Plus – supportive care

Back
ACUTE
|
HIV-associated KS
|
with refractory or relapsed KS
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

classic (sporadic) KS

1st line – local therapy

Back
ACUTE
|
classic (sporadic) KS
|
with localized lesion(s)
1st line – 

local therapy

There is no standard therapy for classic KS. Treatment must be tailored to the individual patient.

Local therapy can be considered for individual cutaneous lesions (symptomatic and/or cosmetically disturbing) and for patients unable to tolerate systemic therapy.[5]​ Options include: topical therapies (e.g., retinoids such as alitretinoin, or imiquimod); radiation therapy; intralesional chemotherapy (vinblastine or bleomycin); cryotherapy; marginal excision; and electrodesiccation and curettage.[2][3][5]

Alitretinoin is self-administered and patients usually require at least 4-8 weeks of treatment. Imiquimod is also self-administered. It requires at least 24 weeks of treatment, and is usually applied under occlusion for 10-12 hours at a time. Dermal irritation at the site of application is common.

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.[2] 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]

Primary options

alitretinoin topical: (0.1%) apply to the affected area(s) twice to four times daily

OR

imiquimod topical: (5%) apply to the affected area(s) three times weekly initially, titrate frequency of application to tolerance and effect up to once daily

OR

vinblastine: consult specialist for guidance on intralesional dose

OR

bleomycin: consult specialist for guidance on intralesional dose

1st line – systemic therapy

Back
ACUTE
|
classic (sporadic) KS
|
with widespread lesions ± systemic involvement
1st line – 

systemic therapy

Indications for systemic treatment include: widespread skin involvement; extensive mucosal lesions; symptomatic edema; rapidly progressive disease; symptomatic visceral disease; and KS flare (exacerbation).[5]

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. Acute infusion reactions are characterized by back pain, shortness of breath, and intense flushing. These reactions typically occur within moments of starting the infusion and subside rapidly after drug discontinuation. Infusions are sometimes able to be resumed at a slower rate.

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. Reduced corticosteroid premedication should be used.

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

See local specialist protocol for dosing guidelines.

Primary options

doxorubicin liposomal

Secondary options

paclitaxel

Tertiary options

paclitaxel nanoparticle albumin-bound

Plus – supportive care

Back
ACUTE
|
classic (sporadic) KS
|
with widespread lesions ± systemic involvement
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

1st line – systemic therapy

Back
ACUTE
|
classic (sporadic) KS
|
with refractory or relapsed KS
1st line – 

systemic therapy

Pomalidomide, bortezomib, gemcitabine, lenalidomide, or vinorelbine can be considered as first-line options for patients with refractory/relapsed KS.[2][62][63]​​ 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 antiretroviral therapy (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 for KS in the refractory/relapsed setting include: etoposide; imatinib (a tyrosine kinase inhibitor); nivolumab (an anti-programmed death-1 [anti-PD-1] monoclonal antibody immune checkpoint inhibitor) with or without ipilimumab (an anticytotoxic T lymphocyte-associated protein-4 [CTLA-4] monoclonal antibody immune checkpoint inhibitor); pembrolizumab (an anti-PD-1 monoclonal antibody immune checkpoint inhibitor).[2][70][73][74]

Immune checkpoint inhibitors should not be used in patients with concomitant multicentric Castleman disease (MCD) or KS inflammatory cytokine syndrome (KICS) 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.

See local specialist protocol for dosing guidelines.

Primary options

pomalidomide

OR

bortezomib

OR

gemcitabine

OR

lenalidomide

OR

vinorelbine

Secondary options

etoposide

OR

imatinib

OR

nivolumab

OR

nivolumab

and

ipilimumab

OR

pembrolizumab

Plus – supportive care

Back
ACUTE
|
classic (sporadic) KS
|
with refractory or relapsed KS
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

iatrogenic (transplant-related) KS

1st line – manipulation of immunosuppressants

Back
ACUTE
|
iatrogenic (transplant-related) KS
1st line – 

manipulation of immunosuppressants

For patients with iatrogenic KS, careful reduction of immunosuppressive therapy should be considered.

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 difficult in patients with heart or liver transplants.

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]

Consider – local therapy

Back
ACUTE
|
iatrogenic (transplant-related) KS
|
with localized lesion(s)
Consider – 

local therapy

Treatment recommended for SOME patients in selected patient group

Considered for individual cutaneous lesions (symptomatic and/or cosmetically disturbing) and for patients unable to tolerate systemic therapy.[5]​ Options include: topical therapies (e.g., retinoids such as alitretinoin, or imiquimod); radiation therapy; intralesional chemotherapy (vinblastine or bleomycin); cryotherapy; marginal excision; and electrodesiccation and curettage.[2][3][5]

Alitretinoin is self-administered and patients usually require at least 4-8 weeks of treatment. Imiquimod is also self-administered. It requires at least 24 weeks of treatment, and is usually applied under occlusion for 10-12 hours at a time. Dermal irritation at the site of application is common.

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.[2] 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]

Primary options

alitretinoin topical: (0.1%) apply to the affected area(s) twice to four times daily

OR

imiquimod topical: (5%) apply to the affected area(s) three times weekly initially, titrate frequency of application to tolerance and effect up to once daily

OR

vinblastine: consult specialist for guidance on intralesional dose

OR

bleomycin: consult specialist for guidance on intralesional dose

Plus – systemic therapy

Back
ACUTE
|
iatrogenic (transplant-related) KS
|
with widespread lesions ± systemic involvement
Plus – 

systemic therapy

Treatment recommended for ALL patients in selected patient group

Indications for systemic treatment include: widespread mucocutaneous skin involvement; extensive mucosal lesions; symptomatic edema; rapidly progressive disease; symptomatic visceral disease; and KS flare (exacerbation).[5]​ The systemic treatments used for classic and HIV-associated KS can be used for iatrogenic (transplant-associated) KS, given alongside sirolimus for aggressive or advanced disease.[2][3][5]

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. Acute infusion reactions are characterized by back pain, shortness of breath, and intense flushing. These reactions typically occur within moments of starting the infusion and subside rapidly after drug discontinuation. Infusions are sometimes able to be resumed at a slower rate.

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. Reduced corticosteroid premedication should be used.

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

See local specialist protocol for dosing guidelines.

Primary options

doxorubicin liposomal

Secondary options

paclitaxel

Tertiary options

paclitaxel nanoparticle albumin-bound

Plus – supportive care

Back
ACUTE
|
iatrogenic (transplant-related) KS
|
with widespread lesions ± systemic involvement
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

Plus – systemic therapy

Back
ACUTE
|
iatrogenic (transplant-related) KS
|
with refractory or relapsed KS
Plus – 

systemic therapy

Treatment recommended for ALL patients in selected patient group

Pomalidomide, bortezomib, gemcitabine, lenalidomide, or vinorelbine can be considered as first-line options for patients with refractory/relapsed KS.[2][62][63]​ 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 antiretroviral therapy (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 for KS in the refractory/relapsed setting include: etoposide; imatinib (a tyrosine kinase inhibitor); nivolumab (an anti-programmed death-1 [anti-PD-1] monoclonal antibody immune checkpoint inhibitor) with or without ipilimumab (an anticytotoxic T lymphocyte-associated protein-4 [CTLA-4] monoclonal antibody immune checkpoint inhibitor); pembrolizumab (an anti-PD-1 monoclonal antibody immune checkpoint inhibitor).[2]

Immune checkpoint inhibitors should not be used in patients with concomitant multicentric Castleman disease (MCD) or KS inflammatory cytokine syndrome (KICS) 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.

See local specialist protocol for dosing guidelines.

Primary options

pomalidomide

OR

bortezomib

OR

gemcitabine

OR

lenalidomide

OR

vinorelbine

Secondary options

etoposide

OR

imatinib

OR

nivolumab

OR

nivolumab

and

ipilimumab

OR

pembrolizumab

Plus – supportive care

Back
ACUTE
|
iatrogenic (transplant-related) KS
|
with refractory or relapsed KS
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

endemic (observed in sub-Saharan Africa) KS

1st line – local therapy

Back
ACUTE
|
endemic (observed in sub-Saharan Africa) KS
|
with localized lesion(s)
1st line – 

local therapy

Considered for individual cutaneous lesions (symptomatic and/or cosmetically disturbing) and for patients unable to tolerate systemic therapy.[5]​ Options include: topical therapies (e.g., retinoids such as alitretinoin, or imiquimod); radiation therapy; intralesional chemotherapy (vinblastine or bleomycin); cryotherapy; marginal excision; and electrodesiccation and curettage.[2][3][5]

Alitretinoin is self-administered and patients usually require at least 4-8 weeks of treatment. Imiquimod is also self-administered. It requires at least 24 weeks of treatment, and is usually applied under occlusion for 10-12 hours at a time. Dermal irritation at the site of application is common.

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.[2] 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]

Primary options

alitretinoin topical: (0.1%) apply to the affected area(s) twice to four times daily

OR

imiquimod topical: (5%) apply to the affected area(s) three times weekly initially, titrate frequency of application to tolerance and effect up to once daily

OR

vinblastine: consult specialist for guidance on intralesional dose

OR

bleomycin: consult specialist for guidance on intralesional dose

1st line – systemic therapy

Back
ACUTE
|
endemic (observed in sub-Saharan Africa) KS
|
with widespread lesions ± systemic involvement
1st line – 

systemic therapy

Indications for systemic treatment include: widespread skin involvement; extensive mucosal lesions; symptomatic edema; rapidly progressive disease; symptomatic visceral disease; and KS flare (exacerbation).[5]

Liposomal doxorubicin is used 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. Acute infusion reactions are characterized by back pain, shortness of breath, and intense flushing. These reactions typically occur within moments of starting the infusion and subside rapidly after drug discontinuation. Infusions are sometimes able to be resumed at a slower rate.

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. Reduced corticosteroid premedication should be used.

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

See local specialist protocol for dosing guidelines.

Primary options

doxorubicin liposomal

Secondary options

paclitaxel

Tertiary options

paclitaxel nanoparticle albumin-bound

Plus – supportive care

Back
ACUTE
|
endemic (observed in sub-Saharan Africa) KS
|
with widespread lesions ± systemic involvement
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

1st line – systemic therapy

Back
ACUTE
|
endemic (observed in sub-Saharan Africa) KS
|
with refractory or relapsed KS
1st line – 

systemic therapy

Pomalidomide, bortezomib, gemcitabine, lenalidomide, or vinorelbine can be considered as first-line options for patients with refractory/relapsed KS.[2][62]​​[63]​ 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 antiretroviral therapy (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 for KS in the refractory/relapsed setting include: etoposide; imatinib (a tyrosine kinase inhibitor); nivolumab (an anti-programmed death-1 [anti-PD-1] monoclonal antibody immune checkpoint inhibitor) with or without ipilimumab (an anticytotoxic T lymphocyte-associated protein-4 [CTLA-4] monoclonal antibody immune checkpoint inhibitor); pembrolizumab (an anti-PD-1 monoclonal antibody immune checkpoint inhibitor).[2][73]

Immune checkpoint inhibitors should not be used in patients with concomitant multicentric Castleman disease (MCD) or KS inflammatory cytokine syndrome (KICS) 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.

See local specialist protocol for dosing guidelines.

Primary options

pomalidomide

OR

bortezomib

OR

gemcitabine

OR

lenalidomide

OR

vinorelbine

Secondary options

etoposide

OR

imatinib

OR

nivolumab

OR

nivolumab

and

ipilimumab

OR

pembrolizumab

Plus – supportive care

Back
ACUTE
|
endemic (observed in sub-Saharan Africa) KS
|
with refractory or relapsed KS
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

KS with systemic involvement, particularly visceral and lymph node involvement, can carry a poor prognosis. Supportive care may be required for a patient's nutritional, mobility, social, and psychological wellbeing, to control pain, and to monitor and treat disease complications if they occur.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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