What is Kaposi Sarcoma
Kaposi sarcoma (KS) is a type of cancer that develops in the lining of blood and lymph vessels and can affect the skin, mucous membranes, and internal organs. Primarily it appears as painless purplish spots on legs, feet, and face, but later on can extend to the genital area, mouth, or lymph nodes. The lesions (tumors) can also appear in the gestro-intestinal tract–from mouth to anal opening. It is most commonly seen in people with compromised immune systems, such as HIV/AIDS patients or organ transplant recipients, but it can also occur in people with healthy immune systems, especially in those of Eastern European or Mediterranean descent. The cancer is caused by the human herpesvirus 8 (HHV8), also known as Kaposi sarcoma-associated herpesvirus (KSHV).
The symptoms of KS vary depending on the location and severity of cancer. It may cause purple or red patches on the skin, lumps or sores on the mucous membranes, or swelling in the legs. In some cases, it may also cause internal organ damage or failure.
Treatment for KS may include chemotherapy, radiation therapy, immune system-boosting medications, or surgery to remove the affected tissues. The outlook for KS depends on the stage of cancer and the overall health of the patient. In people with compromised immune systems, early diagnosis and treatment are important to improve the chances of a positive outcome.
Symptoms and signs of Kaposi Sarcoma
Kaposi sarcoma is a type of cancer that can affect the skin, mucous membranes, and lymph nodes. It is caused by infection with the human herpesvirus 8 (HHV-8). The most common symptoms of Kaposi sarcoma are:
- Purple or red patches or bumps on the skin
- Swelling in the legs or arms
- Lumps on the skin or in the mouth
- Swelling in the lymph nodes
- Trouble breathing
- Coughing up blood
If you have any of these symptoms, it is important to see a doctor as soon as possible. Kaposi sarcoma can be treated with medications, radiation therapy, and/or surgery. Early diagnosis and treatment can help improve the chances of a successful outcome.
Causes of Kaposi Sarcoma
Human Herpesvirus 8 (HHV-8) is the primary cause of Kaposi Sarcoma. HH-8 is a rare form of cancer that affects the skin, lymph nodes, and internal organs. HHV-8 is most commonly found in people with weakened immune systems, such as those with HIV/AIDS or organ transplant recipients. HHV-8 is present in our body, but it remains quite harmless, as it is suppressed by our immune system. In case of a compromised immune system, HHV-8 causes its harboring cells to divide rapidly. Therefore, in persons with weakened immune systems, the Virus HHV-8 potentially triggers Kaposi Sarcoma. There are 3 main reasons which may trigger it;
- People with HIV (AIDS): Kaposi Sarcoma is caused by a virus called Kaposi Sarcoma-associated herpesvirus (KSHV), which is found in the saliva, urine, and blood of people with HIV. When the immune system is weakened by HIV infection, the virus can multiply and cause Kaposi Sarcoma.
- People with Transplanted Organs: It is possible for a person who has received an organ transplant to develop Kaposi Sarcoma. This is because organ transplant recipients are often required to take immunosuppressive drugs to prevent rejection of the transplanted organ. These drugs can weaken the immune system, making the person more susceptible to infections and cancer.
- Elderly Europeans and Classic Kaposi Sarcoma: In the past, classic KS was most commonly seen in elderly European men of Ashkenazi Jewish descent. However, with the advent of more effective treatments for HIV/AIDS, the incidence of classic KS has decreased significantly in this population.Today, classic KS is still more common in older individuals and is often seen in people over the age of 60, regardless of their ethnicity or geographical location. It is also more commonly seen in men than women.
Diagnosis of Kaposi Sarcoma
In order to determine, whether the suspicious patient, having lesions similar to K.S., has Kaposi Sarcoma or not, the doctor performs a biopsy in the laboratory. A biopsy involves removing a small piece of tissue for examination in a laboratory. Following tests are conducted to determine the status of Kaposi Sarcoma;
- Physical examination: A doctor will visually inspect the affected area and feel for any abnormalities or lumps.
- Blood Tests: Blood tests can check for certain proteins or markers that may indicate the presence of Kaposi sarcoma.
- Fecal occult blood test: Fecal occult blood test is done to detect hidden blood cells in stools. The fecal occult blood test is not typically used to diagnose all kinds of Kaposi Sarcoma. However, it is helpful to detect the signs of Kaposi Sarcoma in the digestive tract.
- Chest X-ray: Chest X-ray is helpful only if Kaposi Sarcoma has affected the internal organs. If Kaposi Sarcoma affected the lungs, the chest x-ray will reveal abnormalities in the chest.
- Bronchoscopy: A bronchoscopy is a procedure that allows a doctor to examine the inside of the bronchial tubes and lungs using a thin, flexible tube called a bronchoscope. Bronchoscopy is unnecessary for diagnosis of Kaposi’s sarcoma unless you have unexplained breathing problems or an abnormal chest X-ray.During the procedure, the doctor will insert the bronchoscope through the nose or mouth and into the bronchial tubes. The bronchoscope has a light and a camera, which allows the doctor to see inside the bronchial tubes and take samples of any abnormalities.If the doctor sees any abnormalities in the bronchial tubes, they may take a biopsy (a small sample of tissue) to send to a laboratory for further testing. This can help confirm a diagnosis of Kaposi sarcoma.
- Endoscopy of Esophagus: An upper endoscopy is a procedure in which a thin, flexible tube with a light and camera on the end is inserted through the mouth and into the esophagus, stomach, and upper part of the small intestine. The camera allows the doctor to see inside the body and check for any abnormalities.During an upper endoscopy, the doctor will be able to see any abnormalities in the esophagus, stomach, and upper part of the small intestine. If the doctor sees any signs of Kaposi Sarcoma, they may take a biopsy (a small sample of tissue) to confirm the diagnosis.
- Colonoscopy: A colonoscopy is a diagnostic procedure that involves inserting a flexible tube with a camera attached to the colon through the anus. This procedure allows the doctor to examine the inside of the colon and rectum for any abnormalities or signs of disease.While a colonoscopy is not specifically used to diagnose Kaposi sarcoma, it can be helpful in identifying any abnormal growths or tumors in the colon and rectum. If the doctor notices any suspicious areas during the colonoscopy, they may recommend further testing or biopsy to determine if it is Kaposi sarcoma or another type of cancer.
- Immunohistochemistry: This test involves applying a special dye to a tissue sample and examining it under a microscope to identify the presence of certain proteins or markers that may indicate Kaposi sarcoma.
Treatment of Kaposi Sarcoma
Treatment of Kaposi Sarcoma varies depending on the following factors;
- Type of disease. Historically, AIDS-related Kaposi’s sarcoma has been more serious than the classic or transplant-related disease. Thanks to increasingly effective antiviral drug combinations and improved prevention of other AIDS-related infections, Kaposi’s sarcoma has become less common and less severe in people with AIDS. Antiretroviral therapy (ART)is helpful if the patient has HIV/AIDS. ART may be used to suppress the virus and improve the immune system.
- Number and location of lesions. Widespread skin lesions and internal lesions require different treatments from isolated lesions. If the lesions are scattered and less in number they can be removed by simple surgery. On the other hand, if they are large in number and scattered all through the body, chemotherapy, and radiotherapy may be helpful.
- Effects of the lesions. Lesions in the mouth and throat make eating difficult, while lesions in the lung can cause shortness of breath. Large lesions, particularly on the upper legs, can lead to painful swelling and difficulty moving around.
- General health. The immune system impairment that makes you vulnerable to Kaposi’s sarcoma also makes certain treatments, such as powerful chemotherapy drugs, too risky to try. The same is true if you also have another type of cancer, poorly controlled diabetes or any serious, chronic disease.
For AIDS-related Kaposi’s sarcoma, the first step in treatment is to start or switch to an antiviral drug combination that will reduce the amount of the virus that causes HIV/AIDS and increase the number of certain immune cells in your body. Sometimes, this is the only treatment needed.
When possible, people with transplant-related Kaposi’s sarcoma may be able to stop taking immune system-suppressing medication. This allows the immune system to eliminate the cancer in some cases. Switching to a different immunosuppressive medication can also bring improvement.
Treatment of Kaposi Sarcoma may include;
- Antiretroviral therapy (ART): If the patient has HIV/AIDS, ART may be used to suppress the virus and improve the immune system.
- Chemotherapy: Chemotherapy drugs may be used to kill cancer cells and shrink tumors.
- Radiotherapy: High-energy rays may be used to kill cancer cells and shrink tumors.
- Surgery: In some cases, surgery may be needed to remove tumors or affected areas of the skin or internal organs.
- Immunotherapy: This treatment uses the patient’s own immune system to fight the cancer cells.
- Topical treatment: Creams or ointments may be used to treat skin lesions.
It is important to work with a healthcare team to determine the best treatment plan for an individual patient. Lesions treated in any of these ways are likely to return within a couple of years. When this happens, treatment can often be repeated.
Radiation is the usual treatment for those with multiple skin lesions. The type of radiation used and the locations of lesions being treated vary from person to person. When more than 25 lesions are present, chemotherapy with standard anti-cancer drugs may be helpful. Chemotherapy is also used to treat Kaposi’s sarcoma in the lymph nodes and digestive tract.
Complications associated with Kaposi Sarcoma
Some possible complications of Kaposi Sarcoma include:
- Pain: Tumors can cause pain, especially if they are located in areas that experience pressure or movement, such as the feet or hands.
- Ulceration: Tumors can break open and cause sores or ulcers on the skin, which can be prone to infection.
- Bleeding: Kaposi Sarcoma can cause abnormal bleeding, especially in the gastrointestinal tract, which can lead to anemia or other health problems.
- Disfigurement: Tumors can grow on the face or other visible areas of the body, causing disfigurement and distress.
- Compromised immune system: Kaposi Sarcoma is often associated with HIV or AIDS, which can weaken the immune system and make individuals more prone to other infections and illnesses.
- Spread to other organs: In advanced stages, Kaposi Sarcoma can spread to other organs in the body, such as the liver, lungs, or lymph nodes, leading to additional complications and a poor prognosis.