Symptoms of Kaposi's sarcoma (KS), a rare cancer seen mainly with HIV, are painless, purplish lesions on the skin, mouth, lymph nodes, and internal organs. Kaposi's sarcoma (KS) is a type of cancer that can form purplish masses (tumors) on the skin, in the mouth, in lymph nodes, and in other organs. There are different types of KS, some of which cause tumors to develop and
Kaposi's sarcoma (KS) is a type of cancer that can form purplish masses (tumors) on the skin, in the mouth, in lymph 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 with advanced human immunodeficiency virus (HIV) infection, but there are other types that can affect groups of people without HIV.
The common thread that connects all of these types is a weakened immune system coupled with an infection by a virus known as human herpesvirus 8 (HHV8). The underlying cause of a weakened immune system largely determines how aggressively or slowly KS symptoms develop.
This article looks at the common signs and symptoms of Kaposi's sarcoma and takes a deeper dive into how the symptoms and complications vary by the different KS types.
Kaposi's sarcoma is a rare type of cancer known as a sarcoma that causes the formation of tumors in soft tissue, such as bones, tendons, muscles, ligaments, cartilage, and fat.
With KS, the disease develops from cells called endothelial cells that line the blood vessels. It is considered angioproliferative because 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 with AIDS, the most advanced stage of HIV infection.
In people with AIDS, the immune system has been so weakened that a person is considered immunocompromised (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 the lymphatic 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.
The three organs of the lymphatic system most commonly affected by KS are:
The complications and symptoms of Kaposi's sarcoma can vary by the type of KS a person has. The four broad categories are:
With HIV-associated KS, tumors can spread to different organs of the body, such as the digestive tract, lungs, liver, and heart.
The symptoms can vary by which organ systems are involved, including:
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.
The underlying cause of transplant-related KS is the use of immunosuppressant drugs to prevent organ transplant rejection. Transplant-related KS can occur with any solid organ transplant but is most likely to occur after a lung transplant or kidney 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.
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.
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.
Being immunocompromised means you are vulnerable to an array of infections (called opportunistic infections) that your body would otherwise be able to fight off. The only way to prevent these is by starting antiretroviral therapy, which suppresses and controls HIV and allows your immune system to rebuild itself and become stronger.
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 evaluated to determine the underlying cause.
Kaposi sarcoma is always a big deal.
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.
According to a 2021 study in the journal Cancer, more and more people on antiretroviral therapy are developing KS even when their viral loads are fully undetectable, indicating that the virus is fully under control.
What the researchers found was that, even with effective HIV treatment, people are still vulnerable to KS if their immune systems are not approaching normal levels (as measured by the CD4 count). Delaying treatment until the CD4 count is low makes it more difficult to rebuild the immune system to a "safe" level. Some people who start late never do.
So, if you are at risk of HIV and do not know your status, talk to your healthcare provider about getting an HIV test. Even if you test positive for the virus, early treatment can better ensure that you live a long, healthy, disease-free life.Symptoms of Kaposi's Sarcoma View Story