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Kaposi sarcoma

aka KS, HHV-8-associated sarcoma

A blood-vessel cancer caused by a herpesvirus, made famous by the AIDS epidemic. In people with HIV, antiretroviral therapy alone often shrinks it; chemotherapy such as liposomal doxorubicin or paclitaxel treats advanced disease, and it remains a major cancer in Africa.

Kaposi sarcoma is a KSHV/HHV-8-driven vascular tumour with four epidemiologic forms: classic (elderly Mediterranean/Eastern European men, indolent), endemic African (including an aggressive lymphadenopathic childhood form), iatrogenic (transplant immunosuppression), and epidemic (AIDS-associated), plus KS in men who have sex with men with controlled HIV. Lesions involve skin, mucosa, lymph nodes and viscera (lung, gut); KSHV also causes primary effusion lymphoma and multicentric Castleman disease, and KS inflammatory cytokine syndrome (KICS).

AIDS-KS: antiretroviral therapy is the foundation and suffices for limited disease; advanced disease (visceral, oedema, rapid progression, T1 by ACTG staging) adds pegylated liposomal doxorubicin (first line) or paclitaxel, both approved in the 1990s; pomalidomide (2020) was the first new KS drug in two decades and works in HIV-positive and -negative patients. Iatrogenic KS responds to reducing immunosuppression or switching to mTOR inhibitors (sirolimus). Classic KS is treated with local therapy (radiotherapy, intralesional vincristine, cryotherapy) or the same systemic agents. In Africa, where paclitaxel is often unaffordable, bleomycin-vincristine regimens remain in use and ACTG A5263 showed paclitaxel superior to oral etoposide and BV. Immune checkpoint inhibitors show activity in small series.

State of the art today

  • Pomalidomide (2020) is the first new approved KS drug since 1997 and the first that also covers HIV-negative KS.
  • Africa carries the burden: KS is a top-five cancer in many countries, and access to paclitaxel or liposomal doxorubicin is the limiting factor.
  • Immunotherapy (PD-1) shows activity and is being tested; KSHV-targeted therapy remains investigational.
Show survival figures (1)

Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.

  • ART transformed AIDS-KS from a leading cause of death into a treatable condition in high-income countries; incidence fell >80% after 1996.
Who it affects

About 35,000 cases per year worldwide, most in sub-Saharan Africa where it is among the commonest cancers; caused by Kaposi sarcoma herpesvirus (KSHV/HHV-8), with HIV the major cofactor.

Group
skin

Where the cases are

Cases by country · GLOBOCAN 2022
All countries →

No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.

Standard of care

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AIDS-KS, limited (T0)

Antiretroviral therapy; observe for regression (watch for IRIS-KS flare); local therapy for cosmetically or functionally important lesions.

NCCN · Category 2A
IMRT / IGRT (modern external beam)
AIDS-KS, advanced (T1) or symptomatic

ART plus pegylated liposomal doxorubicin (preferred) or paclitaxel; pomalidomide as an oral option; continue until maximal response.

NCCN · Category 1 (PLD, paclitaxel); 2A (pomalidom…
Pegylated liposomal doxorubicinPaclitaxel / nab-paclitaxelPomalidomide
Classic or HIV-negative KS

Local radiotherapy, intralesional vincristine or cryotherapy for few lesions; pegylated liposomal doxorubicin, paclitaxel or pomalidomide for extensive disease.

NCCN · Category 2A
Pegylated liposomal doxorubicinPaclitaxel / nab-paclitaxelPomalidomideVincristine
Iatrogenic KS

Reduce immunosuppression; switch calcineurin inhibitor to sirolimus/everolimus; chemotherapy if progressive.

Everolimus
Resource-limited settings

ART plus paclitaxel where available (ACTG A5263); bleomycin-vincristine otherwise; task-shifted oncology nursing models.

ACTG A5263 (Lancet 2020)
Paclitaxel / nab-paclitaxelBleomycinVincristine

Subtypes & biomarkers

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Subtypes
  • Classic (sporadic) KS
  • Endemic African KS (including lymphadenopathic childhood form)
  • Iatrogenic (post-transplant) KS
  • Epidemic (AIDS-associated) KS
  • KS in HIV-negative MSM
  • KSHV-associated : primary effusion lymphoma, multicentric Castleman disease, KICS
Biomarkers clinicians test

Target prevalence in this cancer

History

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  1. 1872Moritz Kaposi describes 'idiopathic multiple pigmented sarcoma of the skin'
  2. 1981KS in young gay men heralds the AIDS epidemic (CDC MMWR)
  3. 1994KSHV/HHV-8 discovered (Chang and Moore, Science)
  4. 1995Liposomal doxorubicin approved for AIDS-KS
    Pegylated liposomal doxorubicin
  5. 1996Combination ART causes KS regression; incidence collapses
  6. 1997Paclitaxel approved for AIDS-KS
    Paclitaxel / nab-paclitaxel
  7. 2020Pomalidomide approved for KS (HIV-positive and -negative)
    Pomalidomide
  8. 2020ACTG A5263: paclitaxel superior to oral etoposide and bleomycin-vincristine in Africa (Lancet)
    Paclitaxel / nab-paclitaxelBleomycin

Pipeline

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Not mapped hereOral cereblon E3 ligase modulator (IMiD)
Pomalidomide · Pomalyst / Imnovid
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
ApprovedMonoclonal antibody (anti-PD-1)
Nivolumab · Opdivo / Opdivo Qvantig (SC)

Open problems

  • Access to effective chemotherapy and ART-linked cancer care in sub-Saharan Africa.
  • KS in people with suppressed HIV and normal CD4 counts (unexplained).
  • No antiviral therapy targets latent KSHV.
  • Endemic childhood KS in Africa remains lethal.

Trials

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Recruiting now (live from ClinicalTrials.gov)

Recruiting trials near you · live from ClinicalTrials.gov
Kaposi sarcoma
condition: Kaposi sarcoma
Open on ClinicalTrials.gov →

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Expert centres

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Where the expertise is

Centres linked to this cancer in OnCo

Seeking a second opinion: ask your oncologist for a referral to a high-volume centre; most accept records and pathology by mail or telehealth. In the US, use the NCI's Find a Cancer Center tool or the nonprofit Cancer Commons, which navigates options for advanced cancers at no cost.

Questions to ask

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Bring to your appointment

Questions to ask your oncologist about Kaposi sarcoma

Generated from this cancer's standard of care, biomarkers, and pipeline · 18 questions

Newly diagnosed

  1. What is my exact diagnosis, stage, and grade, and which tests established them?
    Why: Everything else follows from an accurate stage and subtype.
  2. Which biomarkers have been tested on my tumour (for example HHV-8 LANA-1 immunohistochemistry, HIV status, CD4 count, viral load, ACTG TIS staging, KSHV viral load, Visceral involvement), and what were the results?
    Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
  3. Which subtype is my cancer, and does that change the recommended treatment?
    Why: Recognised subtypes for this cancer include ClassicKS, Endemic African KS, IatrogenicKS.
  4. Is germline (inherited) genetic testing recommended for me or my family?
    Why: Inherited variants can change treatment and matter for relatives.

AIDS-KS, limited (T0)

  1. For my situation (aids-ks, limited (t0)), which of the standard options do you recommend and why?
    Why: Guideline options include: Antiretroviral therapy; observe for regression (watch for IRIS-KS flare); local therapy for cosmetically or functionally important lesions.

AIDS-KS, advanced (T1) or symptomatic

  1. For my situation (aids-ks, advanced (t1) or symptomatic), which of the standard options do you recommend and why?
    Why: Guideline options include: ART plus pegylated liposomal doxorubicin (preferred) or paclitaxel; pomalidomide as an oral option; continue until maximal response.
  2. Am I a candidate for Pegylated liposomal doxorubicin, Paclitaxel / nab-paclitaxel, Pomalidomide, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Classic or HIV-negative KS

  1. For my situation (classic or hiv-negative ks), which of the standard options do you recommend and why?
    Why: Guideline options include: Local radiotherapy, intralesional vincristine or cryotherapy for few lesions; pegylated liposomal doxorubicin, paclitaxel or pomalidomide for extensive disease.
  2. Am I a candidate for Pegylated liposomal doxorubicin, Paclitaxel / nab-paclitaxel, Pomalidomide or related drugs, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Iatrogenic KS

  1. For my situation (iatrogenic ks), which of the standard options do you recommend and why?
    Why: Guideline options include: Reduce immunosuppression; switch calcineurin inhibitor to sirolimus/everolimus; chemotherapy if progressive.
  2. Am I a candidate for Everolimus, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Resource-limited settings

  1. For my situation (resource-limited settings), which of the standard options do you recommend and why?
    Why: Guideline options include: ART plus paclitaxel where available (ACTG A5263); bleomycin-vincristine otherwise; task-shifted oncology nursing models.
  2. Am I a candidate for Paclitaxel / nab-paclitaxel, Bleomycin, Vincristine, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Any stage

  1. Are there clinical trials I could join, for example of Pomalidomide, Pembrolizumab, Nivolumab?
    Why: Trials are how the next standard of care is set; asking early keeps options open.
  2. Would a second opinion at a high-volume centre change anything, and can you help arrange it?
    Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
  3. What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
    Why: Supportive care improves quality of life and helps patients complete treatment.
  4. I read that “Access to effective chemotherapy and ART-linked cancer care in sub-Saharan Africa”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.
  5. I read that “KS in people with suppressed HIV and normal CD4 counts (unexplained)”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.

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Everything relevant

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Direct links plus the targets, companies, and technologies of this cancer's products.

technologies

9
Cytokines & engineered cytokinesCytotoxic chemotherapyElectrochemotherapyGlobal oncology and access in low- and middle-income countriesImmune checkpoint inhibitorsIMRT / IGRT (modern external beam)PROTACs & molecular glues (targeted protein degradation)Small-molecule kinase inhibitorsTopoisomerase-I inhibitors (and ADC payloads)

targets

4
AKTPD-1PIK3CA / PI3K-alphaVEGF / VEGFR

drugs

10
Not mapped hereGlycopeptide antibiotic (DNA-cleaving)
Bleomycin · Blenoxane
ApprovedSmall-molecule mTOR inhibitor
Everolimus · Afinitor
HistoricRecombinant type I interferon (cytokine)
Interferon alfa-2a/2b · Roferon-A / Intron A / Sylatron
ApprovedMonoclonal antibody (anti-PD-1)
Nivolumab · Opdivo / Opdivo Qvantig (SC)
ApprovedCytotoxic chemotherapy (taxane)
Paclitaxel / nab-paclitaxel · Taxol / Abraxane
ApprovedCytotoxic (liposomal anthracycline)
Pegylated liposomal doxorubicin · Doxil / Caelyx
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
Not mapped hereOral cereblon E3 ligase modulator (IMiD)
Pomalidomide · Pomalyst / Imnovid
Not mapped hereVinca alkaloid (microtubule inhibitor)
Vinblastine · Velban
Not mapped hereVinca alkaloid (microtubule inhibitor)
Vincristine · Oncovin / Marqibo

companies

4
Bristol Myers SquibbJohnson & JohnsonMerck & Co. (MSD)Novartis

institutions

2
UNC Lineberger Comprehensive Cancer CenterUPMC Hillman Cancer Center

pathways

3
Oncogenic virusesPI3K / AKT / mTORVEGF angiogenesis

terms

1
Immune-related adverse events (irAEs)

Latest papers

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Latest papers · live from Europe PMC
Open in Europe PMC

Query for this cancer: (TITLE:"Kaposi sarcoma" OR ABSTRACT:"Kaposi sarcoma" OR TITLE:"HHV-8-associated sarcoma" OR ABSTRACT:"HHV-8-associated sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Kaposi sarcoma, not a curated reading list.

Connected

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Pages like this

not linked directly; found by shared links

technologies

5
Cytotoxic chemotherapyElectrochemotherapyGlobal oncology and access in low- and middle-income countriesImmune checkpoint inhibitorsIMRT / IGRT (modern external beam)

targets

2
PD-1VEGF / VEGFR

drugs

10
Not mapped hereGlycopeptide antibiotic (DNA-cleaving)
Bleomycin · Blenoxane
ApprovedSmall-molecule mTOR inhibitor
Everolimus · Afinitor
HistoricRecombinant type I interferon (cytokine)
Interferon alfa-2a/2b · Roferon-A / Intron A / Sylatron
ApprovedMonoclonal antibody (anti-PD-1)
Nivolumab · Opdivo / Opdivo Qvantig (SC)
ApprovedCytotoxic chemotherapy (taxane)
Paclitaxel / nab-paclitaxel · Taxol / Abraxane
ApprovedCytotoxic (liposomal anthracycline)
Pegylated liposomal doxorubicin · Doxil / Caelyx
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
Not mapped hereOral cereblon E3 ligase modulator (IMiD)
Pomalidomide · Pomalyst / Imnovid
Not mapped hereVinca alkaloid (microtubule inhibitor)
Vinblastine · Velban
Not mapped hereVinca alkaloid (microtubule inhibitor)
Vincristine · Oncovin / Marqibo

companies

2
Bristol Myers SquibbJohnson & Johnson

institutions

2
UNC Lineberger Comprehensive Cancer CenterUPMC Hillman Cancer Center

pathways

3
Oncogenic virusesPI3K / AKT / mTORVEGF angiogenesis

terms

1
Immune-related adverse events (irAEs)