Table of ContentsView AllTable of ContentsCommon SymptomsRare SymptomsComplicationsWhen to See a Provider
Table of ContentsView All
View All
Table of Contents
Common Symptoms
Rare Symptoms
Complications
When to See a Provider
Kaposi’s sarcoma(KS) is atype of cancerthat can form purplish masses (tumors) on the skin, in the mouth, inlymph nodes, and in other organs. There are different types of KS, some of which cause tumors to develop and spread rapidly, and others that are slow growing and even short-lived.
KS is typically associated withadvanced human immunodeficiency virus (HIV) infection, but there are other types that can affect groups of people without HIV.
Reproduced with permission from ©DermNet NZ www.dermnetnz.org 2022
Kaposi Sarcoma as an AIDS-Defining Condition
Kaposi’s sarcoma is a rare type of cancer known as asarcomathat causes the formation of tumors in soft tissue, such as bones, tendons, muscles, ligaments, cartilage, and fat.
With KS, the disease develops from cells calledendothelial cellsthat line the blood vessels. It is consideredangioproliferativebecause it spreads (proliferates) through blood vessels (with “angio"meaning blood vessels in Greek). While KS usually causes tumors on the skin, it can sometimes spread to internal organs.
The defining features of KS are the characteristic skin tumors that can also affect the gums and mouth. Among the tell-tale signs are:
For some people, KS may be limited to certain parts of the body (such as the lower legs) and never progresses. However, in rare cases, KS can spread from the skin to the internal organs. This scenario is likely in people withAIDS, the most advanced stage of HIV infection.
In people with AIDS, the immune system has been so weakened that a person is consideredimmunocompromised(meaning lacking adequate immune defense). Without these defenses, diseases like cancer can become invasive and infiltrate organs more readily.
The organs most commonly affected are those of thelymphatic system. The lymphatic system is a network of vessels and organs that, among other things, is responsible for trapping and neutralizing disease-causing agents in the body.
Complications/Subgroup Indications
Complications of HIV-Associated KS
With HIV-associated KS, tumors can spread to different organs of the body, such as the digestive tract, lungs, liver, and heart.
Complications of Classic KS
Classic KS is a less severe form of the disease that is associated with the natural decline in immune function among aging people of certain racial or ethnic origins. Males 70 and over of Jewish, Greek, and Italian descent are most predominantly affected.
Unlike HIV-associated KS, classic KS is rarely aggressive and tends to be limited to the lower legs and hands. KS lesions in the mouth or gums are also uncommon. While classic KS rarely spreads to internal organs, it has been known to occur.
Complications of Transplant-Related KS
The underlying cause of transplant-related KS is the use of immunosuppressant drugs to preventorgan transplant rejection. Transplant-related KS can occur with any solid organ transplant but is most likely to occur after alung transplantorkidney transplant.
Transplanted-related KS tends to affect the lower limbs and will typically clear once the immunosuppressant drug is stopped or switched. In some cases, the lesions may spontaneously clear on their own.
Although it is uncommon for transplant-related KS to affect internal organs, it has been known on rare occasions to spread to the lymph nodes, lungs, or heart. If internal organs are affected, the outcomes are generally poor.
Complications of Endemic KS
Endemic Kaposi’s sarcoma is mainly confined to sub-Saharan Africa and comes in two forms: one that affects children under age 10 and the other that affects men between the ages of 20 and 50.
The form affecting men typically grows slowly and causes nodules on the lower limbs that can remain stable for many years or clear spontaneously. Although some cases have been known to spread rapidly, the lesions tend to be confined to the skin and rarely affect internal organs.
The more aggressive form of endemic KS in children typically causes tumors in the lymph nodes, with or without lesions on the skin. When both the skin and internal organs are involved, the risk of death is high, usually occurring within two months.
HIV Statistics You Should Know
When to See a Healthcare Provider
While the appearance of Kaposi’s sarcoma in people with HIV is not an emergency per se, it does require immediate and sometimes aggressive treatment. Although KS can occur in people with intact immune systems, it is more likely than not to be a sign that your immune system is compromised.
Even if HIV is not involved, there is no situation in which KS should not be seen as a matter of urgency. Even if it is indolent,KS is a rare form of cancer that needs to be evaluatedto determine the underlying cause.
Kaposi sarcoma isalwaysa big deal.
How HIV Is Treated
Summary
Kaposi’s sarcoma (KS) is a rare form of cancer that is mainly seen in people living with advanced HIV infection. The defining feature of KS is painless, purplish tumors that develop on the skin and in the mouth.
In some cases, KS can affect internal organs like the lymph nodes, lungs, liver, intestines, and heart. These can lead to an array of potentially severe and even life-threatening complications.
There are other forms of KS that affect people without HIV. Many of these are slow-growing and some may even spontaneously clear on their own without treatment. Others, like an aggressive form of KS affecting African children, are associated with a high risk of death.
How HIV Is Diagnosed
A Word From Verywell
Many people regard Kaposi’s sarcoma as a relic of the AIDS epidemic of the 1980s and 1990s, but it still occurs today, even among people onantiretroviral therapy.
According to a 2021 study in the journalCancer,more and more people on antiretroviral therapy are developing KS even when theirviral loads are fully undetectable, indicating that the virus is fully under control.
So, if you areat risk of HIVand do not know your status, talk to your healthcare provider about getting anHIV test. Even if youtest positivefor the virus, early treatment can better ensure that you live a long, healthy, disease-free life.
Life Expectancy for People Living With HIV
16 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Curtiss P, Strazzulla LC, Friedman-Kien AE.An update on Kaposi’s sarcoma: epidemiology, pathogenesis and treatment.Dermatol Ther (Heidelb).2016;6(4):465–70. doi:10.1007/s13555-016-0152-3Cancer Research UK.Kaposi’s sarcoma.Grabar S, Costagliola D.Epidemiology of Kaposi’s sarcoma.Cancers (Basel).2021;13(22):5692. doi:10.3390/cancers13225692Zoubeidi H, Aydi Z, Daoud F, et al.Kaposi’s sarcoma presenting as lymphadenopathy in an immunocompetent patient.Eur J Case Rep Intern Med.2016;3(7):000493. doi:10.12890/2016_000493Zhao CJ, Ma GZ, Wang YJ, Wang JH.Splenic Kaposi’s sarcoma in a human immunodeficiency virus-negative patient: a case report.World J Clin Cases.2021;9(18):4765–71. doi:10.12998/wjcc.v9.i18.4765Altunay I, Kucukunal A, Demirci GT, Ates B.Variable clinical presentations of classic Kaposi sarcoma in Turkish patients.J Dermatol Case Rep.2012;6(1):8–13. doi:10.3315/jdcr.2012.1088Cahoon EK, Linet MS, Clarke CA, Pawlish KS, Engels EA, Pfeiffer RM.Risk of Kaposi sarcoma after solid organ transplantation in the United States.Int J Cancer. 2018;143(11):2741-8. doi:10.1002/ijc.31735El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2018;13:33. doi:10.1186/s13027-018-0207-4Lee AJ, Brenner L, Mouad B, Monteiro C, Vega KJ, Munoz JC.Gastrointestinal Kaposi’s sarcoma: case report and review of the literature.World J Gastrointest Pharmacol Ther.2015;6(3):89–95. doi:10.4292/wjgpt.v6.i3.89Nwabudike SM, Hemmings s, Paul Y, Habtegegriel Y, Polk O, Mehari A.Pulmonary Kaposi sarcoma: an uncommon cause of respiratory failure in the era of highly active antiretroviral therapy—case report and review of the literature.Case Rep Infect Dis.2016;2016:9354136. doi:10.1155/2016/9354136Van Leer-Greenberg, Kole A, Chawla S.Hepatic Kaposi sarcoma: a case report and review of the literature.World J Hepatol.2017;9(4):171–9. doi:10.4254/wjh.v9.i4.171Makharoblidze E, Goishvili N, Mchedlishvili M, Jangavadze M.Primary Kaposi’s sarcoma of the heart in non-immunodeficient patient: case report and literature review.Diagn Pathol.2015;10:111. doi:10.1186/s13000-015-0354-5Anderson M, Ying T, Wyburn K.Transplant-associated penile Kaposi sarcoma managed with single agent paclitaxel chemotherapy: a case report.BMC Urol.2021;21:87. doi:10.1186/s12894-021-00855-yMcTighe S, Rivard S, Letizia A, Logemann N, Sulit D, Marquart J.Recommendations for counseling and education of service members on endemic African cutaneous Kaposi sarcoma: a case study.Military Med.2018;183:7-8. doi:10.1093/milmed/usx218El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2w018;13:33. doi:10.1186/s13027-018-0207-4Palich R, Makinson A, Veyri M, et al.Kaposi’s sarcoma in virally suppressed people living with HIV: an emerging condition.Cancers (Basel).2021;13(22):5702. doi:10.3390/cancers13225702
16 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Curtiss P, Strazzulla LC, Friedman-Kien AE.An update on Kaposi’s sarcoma: epidemiology, pathogenesis and treatment.Dermatol Ther (Heidelb).2016;6(4):465–70. doi:10.1007/s13555-016-0152-3Cancer Research UK.Kaposi’s sarcoma.Grabar S, Costagliola D.Epidemiology of Kaposi’s sarcoma.Cancers (Basel).2021;13(22):5692. doi:10.3390/cancers13225692Zoubeidi H, Aydi Z, Daoud F, et al.Kaposi’s sarcoma presenting as lymphadenopathy in an immunocompetent patient.Eur J Case Rep Intern Med.2016;3(7):000493. doi:10.12890/2016_000493Zhao CJ, Ma GZ, Wang YJ, Wang JH.Splenic Kaposi’s sarcoma in a human immunodeficiency virus-negative patient: a case report.World J Clin Cases.2021;9(18):4765–71. doi:10.12998/wjcc.v9.i18.4765Altunay I, Kucukunal A, Demirci GT, Ates B.Variable clinical presentations of classic Kaposi sarcoma in Turkish patients.J Dermatol Case Rep.2012;6(1):8–13. doi:10.3315/jdcr.2012.1088Cahoon EK, Linet MS, Clarke CA, Pawlish KS, Engels EA, Pfeiffer RM.Risk of Kaposi sarcoma after solid organ transplantation in the United States.Int J Cancer. 2018;143(11):2741-8. doi:10.1002/ijc.31735El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2018;13:33. doi:10.1186/s13027-018-0207-4Lee AJ, Brenner L, Mouad B, Monteiro C, Vega KJ, Munoz JC.Gastrointestinal Kaposi’s sarcoma: case report and review of the literature.World J Gastrointest Pharmacol Ther.2015;6(3):89–95. doi:10.4292/wjgpt.v6.i3.89Nwabudike SM, Hemmings s, Paul Y, Habtegegriel Y, Polk O, Mehari A.Pulmonary Kaposi sarcoma: an uncommon cause of respiratory failure in the era of highly active antiretroviral therapy—case report and review of the literature.Case Rep Infect Dis.2016;2016:9354136. doi:10.1155/2016/9354136Van Leer-Greenberg, Kole A, Chawla S.Hepatic Kaposi sarcoma: a case report and review of the literature.World J Hepatol.2017;9(4):171–9. doi:10.4254/wjh.v9.i4.171Makharoblidze E, Goishvili N, Mchedlishvili M, Jangavadze M.Primary Kaposi’s sarcoma of the heart in non-immunodeficient patient: case report and literature review.Diagn Pathol.2015;10:111. doi:10.1186/s13000-015-0354-5Anderson M, Ying T, Wyburn K.Transplant-associated penile Kaposi sarcoma managed with single agent paclitaxel chemotherapy: a case report.BMC Urol.2021;21:87. doi:10.1186/s12894-021-00855-yMcTighe S, Rivard S, Letizia A, Logemann N, Sulit D, Marquart J.Recommendations for counseling and education of service members on endemic African cutaneous Kaposi sarcoma: a case study.Military Med.2018;183:7-8. doi:10.1093/milmed/usx218El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2w018;13:33. doi:10.1186/s13027-018-0207-4Palich R, Makinson A, Veyri M, et al.Kaposi’s sarcoma in virally suppressed people living with HIV: an emerging condition.Cancers (Basel).2021;13(22):5702. doi:10.3390/cancers13225702
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Curtiss P, Strazzulla LC, Friedman-Kien AE.An update on Kaposi’s sarcoma: epidemiology, pathogenesis and treatment.Dermatol Ther (Heidelb).2016;6(4):465–70. doi:10.1007/s13555-016-0152-3Cancer Research UK.Kaposi’s sarcoma.Grabar S, Costagliola D.Epidemiology of Kaposi’s sarcoma.Cancers (Basel).2021;13(22):5692. doi:10.3390/cancers13225692Zoubeidi H, Aydi Z, Daoud F, et al.Kaposi’s sarcoma presenting as lymphadenopathy in an immunocompetent patient.Eur J Case Rep Intern Med.2016;3(7):000493. doi:10.12890/2016_000493Zhao CJ, Ma GZ, Wang YJ, Wang JH.Splenic Kaposi’s sarcoma in a human immunodeficiency virus-negative patient: a case report.World J Clin Cases.2021;9(18):4765–71. doi:10.12998/wjcc.v9.i18.4765Altunay I, Kucukunal A, Demirci GT, Ates B.Variable clinical presentations of classic Kaposi sarcoma in Turkish patients.J Dermatol Case Rep.2012;6(1):8–13. doi:10.3315/jdcr.2012.1088Cahoon EK, Linet MS, Clarke CA, Pawlish KS, Engels EA, Pfeiffer RM.Risk of Kaposi sarcoma after solid organ transplantation in the United States.Int J Cancer. 2018;143(11):2741-8. doi:10.1002/ijc.31735El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2018;13:33. doi:10.1186/s13027-018-0207-4Lee AJ, Brenner L, Mouad B, Monteiro C, Vega KJ, Munoz JC.Gastrointestinal Kaposi’s sarcoma: case report and review of the literature.World J Gastrointest Pharmacol Ther.2015;6(3):89–95. doi:10.4292/wjgpt.v6.i3.89Nwabudike SM, Hemmings s, Paul Y, Habtegegriel Y, Polk O, Mehari A.Pulmonary Kaposi sarcoma: an uncommon cause of respiratory failure in the era of highly active antiretroviral therapy—case report and review of the literature.Case Rep Infect Dis.2016;2016:9354136. doi:10.1155/2016/9354136Van Leer-Greenberg, Kole A, Chawla S.Hepatic Kaposi sarcoma: a case report and review of the literature.World J Hepatol.2017;9(4):171–9. doi:10.4254/wjh.v9.i4.171Makharoblidze E, Goishvili N, Mchedlishvili M, Jangavadze M.Primary Kaposi’s sarcoma of the heart in non-immunodeficient patient: case report and literature review.Diagn Pathol.2015;10:111. doi:10.1186/s13000-015-0354-5Anderson M, Ying T, Wyburn K.Transplant-associated penile Kaposi sarcoma managed with single agent paclitaxel chemotherapy: a case report.BMC Urol.2021;21:87. doi:10.1186/s12894-021-00855-yMcTighe S, Rivard S, Letizia A, Logemann N, Sulit D, Marquart J.Recommendations for counseling and education of service members on endemic African cutaneous Kaposi sarcoma: a case study.Military Med.2018;183:7-8. doi:10.1093/milmed/usx218El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2w018;13:33. doi:10.1186/s13027-018-0207-4Palich R, Makinson A, Veyri M, et al.Kaposi’s sarcoma in virally suppressed people living with HIV: an emerging condition.Cancers (Basel).2021;13(22):5702. doi:10.3390/cancers13225702
Curtiss P, Strazzulla LC, Friedman-Kien AE.An update on Kaposi’s sarcoma: epidemiology, pathogenesis and treatment.Dermatol Ther (Heidelb).2016;6(4):465–70. doi:10.1007/s13555-016-0152-3
Cancer Research UK.Kaposi’s sarcoma.
Grabar S, Costagliola D.Epidemiology of Kaposi’s sarcoma.Cancers (Basel).2021;13(22):5692. doi:10.3390/cancers13225692
Zoubeidi H, Aydi Z, Daoud F, et al.Kaposi’s sarcoma presenting as lymphadenopathy in an immunocompetent patient.Eur J Case Rep Intern Med.2016;3(7):000493. doi:10.12890/2016_000493
Zhao CJ, Ma GZ, Wang YJ, Wang JH.Splenic Kaposi’s sarcoma in a human immunodeficiency virus-negative patient: a case report.World J Clin Cases.2021;9(18):4765–71. doi:10.12998/wjcc.v9.i18.4765
Altunay I, Kucukunal A, Demirci GT, Ates B.Variable clinical presentations of classic Kaposi sarcoma in Turkish patients.J Dermatol Case Rep.2012;6(1):8–13. doi:10.3315/jdcr.2012.1088
Cahoon EK, Linet MS, Clarke CA, Pawlish KS, Engels EA, Pfeiffer RM.Risk of Kaposi sarcoma after solid organ transplantation in the United States.Int J Cancer. 2018;143(11):2741-8. doi:10.1002/ijc.31735
El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2018;13:33. doi:10.1186/s13027-018-0207-4
Lee AJ, Brenner L, Mouad B, Monteiro C, Vega KJ, Munoz JC.Gastrointestinal Kaposi’s sarcoma: case report and review of the literature.World J Gastrointest Pharmacol Ther.2015;6(3):89–95. doi:10.4292/wjgpt.v6.i3.89
Nwabudike SM, Hemmings s, Paul Y, Habtegegriel Y, Polk O, Mehari A.Pulmonary Kaposi sarcoma: an uncommon cause of respiratory failure in the era of highly active antiretroviral therapy—case report and review of the literature.Case Rep Infect Dis.2016;2016:9354136. doi:10.1155/2016/9354136
Van Leer-Greenberg, Kole A, Chawla S.Hepatic Kaposi sarcoma: a case report and review of the literature.World J Hepatol.2017;9(4):171–9. doi:10.4254/wjh.v9.i4.171
Makharoblidze E, Goishvili N, Mchedlishvili M, Jangavadze M.Primary Kaposi’s sarcoma of the heart in non-immunodeficient patient: case report and literature review.Diagn Pathol.2015;10:111. doi:10.1186/s13000-015-0354-5
Anderson M, Ying T, Wyburn K.Transplant-associated penile Kaposi sarcoma managed with single agent paclitaxel chemotherapy: a case report.BMC Urol.2021;21:87. doi:10.1186/s12894-021-00855-y
McTighe S, Rivard S, Letizia A, Logemann N, Sulit D, Marquart J.Recommendations for counseling and education of service members on endemic African cutaneous Kaposi sarcoma: a case study.Military Med.2018;183:7-8. doi:10.1093/milmed/usx218
El-Mallawany NK, Villiera J, Kamiyango W, et al.Endemic Kaposi sarcoma in HIV-negative children and adolescents: an evaluation of overlapping and distinct clinical features in comparison with HIV-related disease.Infect Agents Cancer.2w018;13:33. doi:10.1186/s13027-018-0207-4
Palich R, Makinson A, Veyri M, et al.Kaposi’s sarcoma in virally suppressed people living with HIV: an emerging condition.Cancers (Basel).2021;13(22):5702. doi:10.3390/cancers13225702
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