A rare type of soft tissue sarcoma that causes cancerous spots on the skin and in the tissue that lines the gastrointestinal (GI) tract is called Kaposi sarcoma. While it happens rarely, people with human herpesvirus 8 (HHV-8) and weakened immune systems are more likely to develop it. Generally, this type of cancer is treatable, but it may return after a period.
In other words, Kaposi sarcoma causes lesions that often form on the skin or mucous membranes that line the mouth, nose, and anus. Sometimes, tumors may develop on the lining of certain internal organs, including the liver, abdomen, and lungs.
Generally, this type of cancer occurs quite rarely. Nowadays, there are fewer than 5,000 people with Kaposi sarcoma in the U.S.
Types of Kaposi Sarcoma
Healthcare professionals have divided this condition into 4 categories. For example:
- Epidemic Kaposi sarcoma – This is one of the most common forms of this condition, and it is often related to HIV/AIDS.
- Classic Kaposi sarcoma – This type of Kaposi sarcoma mostly affects males over 60 years old who are of Mediterranean, Southwest Asian, Eastern European, or Ashkenazi Jewish descent. The classic form also tends to spread to internal organs.
- Endemic Kaposi sarcoma – Those who develop this type of cancer often live near the African equator. While it is very similar to the classic form, it appears at a younger age (under 40).
- Acquired Kaposi sarcoma – This type of cancer often occurs in people who carry HHV-8 and administer immunosuppressants.
What Are The Symptoms of Kaposi Sarcoma?
Those who develop this type of cancer may experience different symptoms because it depends on the exact location of the tumor. Check some general symptoms of Kaposi sarcoma below:
- Abnormal spots on the skin (these spots may appear in one or more areas of the body)
- Abdominal pain
- Bloody stools
- Lymphedema (it occurs when a tumor blocks one of the lymph nodes)
- Mouth pain
- Coughing up blood
- Breathing problems
- Constipation
- Diarrhea
- Vomiting
If you experience any of the previous symptoms, do not hesitate to visit a doctor for a diagnosis and treatment. Otherwise, it may lead to serious complications.
Causes and Risk Factors
Typically, Kaposi sarcoma occurs in people who take immunosuppressants and carry HHV-8, which infects the cells that line the blood and lymphatic vessels. Furthermore, healthcare professionals have identified some factors that could make you more prone to Kaposi sarcoma. Check some of them below:
- Age – Most of the time, this type of soft tissue sarcoma is diagnosed in people between the ages of 40 and 70.
- Ethnicity – Those who are of Mediterranean, Southwest Asian, Eastern European, or Ashkenazi Jewish descent are at increased risk of Kaposi sarcoma.
- Immune deficiency – People who administer immunosuppressants or have HIV infection are also at increased risk.
- Location – This type of cancer occurs more frequently in people who live near the equator in Africa.
- Sex – Kaposi sarcoma tends to occur in males more frequently than in females.
- Sexual activity – HHV-8, which is a significant risk factor for Kaposi sarcoma, can spread through unprotected sex. That’s why doctors recommend using condoms or dental dams during sexual activity.
What Are The Possible Complications of Kaposi Sarcoma?
Those who develop Kaposi sarcoma may also experience some complications, especially if the condition is poorly managed. Check some examples below:
- Respiratory distress
- Hemorrhage (uncontrolled bleeding)
- Pneumonitis (inflammation and fluid buildup within the lungs)
- Severe abdominal pain
- GI blockages or intestinal folding
- Lymphedema
- Secondary skin infections
- Ulceration
- Second malignancies (including non-Hodgkin lymphoma)
- Anemia (low red blood cell count)
- KSHV-related disorders – For example, multicentric Castleman disease (MCD), KSHV inflammatory cytokine syndrome (KICS), and others
- Skin abnormalities
The previous list does not contain all possible complications of Kaposi sarcoma. However, you can talk with your physician about ways to reduce the risk or even prevent them. Unfortunately, it is not always possible to reduce the risk of developing Kaposi sarcoma. However, taking steps to reduce the risk of HIV may help prevent this type of cancer, according to the American Cancer Society. Moreover, antiretroviral therapy may help reduce the risk of Kaposi sarcoma in people who already have HIV infection.
Diagnosis
The diagnosis of Kaposi sarcoma begins with a physical examination in which doctors will check your skin for lesions that resemble this type of cancer. They may also ask some questions about your symptoms and medical history to get more clues about your disease. However, to get an accurate diagnosis, physicians usually perform the following tests and procedures. These include:
- Biopsy – During this procedure, physicians will take a sample of affected tissue for testing.
- Bronchoscopy – It helps examine the windpipe and the airways for Kaposi sarcoma.
- Chest X-ray – This is an imaging test used to take images of the lungs.
- CT (computed tomography) scans
- Endoscopy – This test helps doctors examine the stomach and intestines.
- Immunohistochemistry – This is a laboratory test used to check for a specific protein (LANA-1) that is usually associated with HHV-8.
Treatment
Doctors often prescribe different treatments for people with Kaposi sarcoma because they depend on several factors. For example, the type, severity, and cause of the condition, existing health problems, age, gender, and preferences. Check some common treatments below:
- Radiation therapy
- Cryotherapy
- Antiretroviral therapy
- Chemotherapy
- Targeted therapy
- Surgery
Frequently Asked Questions
When should I see my healthcare provider?
Typically, it is recommended to visit a doctor in the following cases. These include when the treatment you follow does not work, Kaposi sarcoma returns after a successful treatment, or you notice worsening of existing symptoms or develop new ones.
How can I take care of myself?
Along with the previous treatment options, doctors may recommend the following tips to maintain a healthy immune system. Check some examples below:
- Manage stress and chronic health conditions (including diabetes, hypertension, hyperlipidemia, and others)
- Stop smoking – If you have problems with smoking cessation, discuss it with your doctor.
- Get plenty of sleep and water
- Stay up to date on vaccines
- Regularly wash your hands with soap and warm water
- Limit or avoid alcoholic drinks
- Adopt a diet full of nutritious foods (including fresh fruits and veggies)
Is Kaposi sarcoma an autoimmune disorder?
No, this condition is a type of soft tissue sarcoma. Most of the time, it occurs in people with weakened immune systems (especially if they carry HHV-8). Autoimmune disorders are conditions in which the immune system attacks healthy cells instead of viruses, bacteria, and other germs that can make you ill.
Can Kaposi sarcoma be cured?
In some cases, Kaposi sarcoma can be controlled or even eliminated with appropriate treatment, especially when diagnosed early. However, because the underlying HHV-8 infection remains in the body, the cancer can recur. Regular follow-up appointments are important to detect any recurrence as early as possible.
Is Kaposi sarcoma contagious?
Kaposi sarcoma itself is not contagious. However, the virus associated with the disease, human herpesvirus 8 (HHV-8), can spread through saliva, sexual contact, blood transfusions, and organ transplantation. Most people infected with HHV-8 never develop Kaposi sarcoma because they have healthy immune systems.
Can people without HIV develop Kaposi sarcoma?
Yes. Although Kaposi sarcoma is strongly associated with HIV/AIDS, it can also occur in people without HIV. For example, it may happen in older adults, people taking immunosuppressive medications after an organ transplant, or individuals living in regions where HHV-8 infection is more common.
What do Kaposi sarcoma skin lesions look like?
Kaposi sarcoma lesions usually appear as flat or raised patches, plaques, or nodules that are red, purple, brown, or bluish in color. They generally do not fade when pressed and may increase in size or number over time. Lesions can develop anywhere on the body, but are most commonly found on the legs, feet, face, and inside the mouth.
Can Kaposi sarcoma spread to other parts of the body?
Yes. Although many cases begin with skin lesions, Kaposi sarcoma can spread to the lymph nodes and internal organs, including the lungs, gastrointestinal tract (GI), and liver. When internal organs are affected, symptoms may include coughing, shortness of breath (dyspnea), abdominal pain, bleeding, or digestive problems.
Does antiretroviral therapy help treat Kaposi sarcoma?
Yes. For people with HIV-associated Kaposi sarcoma, antiretroviral therapy (ART) is one of the most effective treatments because it strengthens the immune system. In some cases, ART alone can cause Kaposi sarcoma lesions to shrink or disappear, while more advanced disease may require additional treatments such as chemotherapy or radiation therapy.
What is the outlook for someone with Kaposi sarcoma?
The prognosis depends on several factors, including the type of Kaposi sarcoma, the strength of the immune system, whether the cancer has spread, and how well it responds to treatment. People with localized disease and well-controlled HIV often have a favorable outlook, while widespread disease or severe immune suppression may require more intensive treatment and closer medical monitoring. If you have any other questions, ask your healthcare provider.
