Kaposi Sarcoma

21.03.2024

DIAGNOSIS AND TREATMENT OF KAPOSI'S SARCOMA

Kaposi sarcoma is a rare type of cancer that affects blood vessels and usually forms lesions on the skin. This cancer occurs when it is caused by a virus called human herpesvirus-8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). Kaposi's sarcoma is more common in people with compromised immune systems, such as HIV/AIDS patients or organ transplant recipients. The lesions are usually purple, red, or brown in color and may appear as patches, plaques, or knots on the skin. These lesions can appear anywhere on the body, but are usually concentrated in areas such as the skin, mouth, lymph nodes, and internal organs. There are four main types of Kaposi's sarcoma: classic (Mediterranean), endemic (African), iatrogenic (after organ transplantation), and epidemic (HIV/AIDS-associated). Treatment depends on the extent of the disease, the patient's general health condition, and other factors that affect the immune system. Treatment options include radiotherapy, chemotherapy and local treatments. The importance of Kaposi's sarcoma has particularly increased during the HIV/AIDS epidemic, and this type of cancer can pose a potentially life-threatening condition in immunocompromised individuals. Therefore, early diagnosis and appropriate treatment are of great importance in controlling the disease and improving the patient's quality of life.

WHAT ARE THE RISK FACTORS?

The main risk factor for Kaposi sarcoma is a weakened immune system. Some risk factors that may increase the risk of developing Kaposi sarcoma:

HIV Infection: The risk of Kaposi's sarcoma is increased in people with Human Immunodeficiency Virus (HIV). Since HIV causes the immune system to weaken, the risk of developing such cancers increases.

Organ transplant: People who have had an organ transplant may be at risk of Kaposi's sarcoma because their immune system is suppressed after the transplant. This development may occur because the immune system is suppressed with immunosuppressive drugs.

Other Immunosuppressive Conditions: The risk of Kaposi sarcoma may be increased in people taking immunosuppressive medications for reasons other than organ transplantation or in those with other immune-suppressing conditions such as autoimmune diseases.

Age: Kaposi sarcoma is more common in older men. The risk is especially higher in people over the age of 60.

Gender: Kaposi sarcoma is more common in men than in women.

Geographical Region: Kaposi's sarcoma is more common in some geographical regions. Especially in Central Africa, Kaposi's sarcoma is a more common type of cancer.

Ethnicity: Kaposi sarcoma is more common, especially in people of Ashkenazi Jewish descent.

Kaposi sarcoma risk factors may vary from person to person, and the combination of these factors may affect the risk of the disease. Especially people with weakened immune systems should pay attention to these risk factors and follow regular health checks.

Image 1: Kaposi sarcoma is a type of cancer that affects blood vessels and causes lesions on the skin.

HOW DOES IT OCCUR?

The mechanisms of Kaposi sarcoma development involve several factors that play a role, especially in people with weakened immune systems. The main cause of this cancer is infection with “Kaposi sarcoma-associated herpesvirus” (KSHV) or “Human Herpes Virus Type 8” (HHV-8), which is the herpes virus associated with Kaposi sarcoma. A weak immune system causes this virus to lose control and genetic changes to occur that can lead to the formation of KSHV cancer cells. Additionally, factors such as using immunosuppressive medications or undergoing an organ transplant may increase the risk of Kaposi's sarcoma because the immune system is suppressed. The mechanisms of development of Kaposi's sarcoma are complex and occur as a result of the interaction of various factors.

WHAT ARE THE SYMPTOMS?

Kaposi sarcoma is a type of cancer that occurs especially in people with weakened immune systems. Symptoms of Kaposi sarcoma may include:

Skin Stains: The most common symptom of Kaposi sarcoma is the appearance of purple, red, or purplish-red spots on the skin. These spots are usually seen in areas such as ankles, legs, face and genital area.

Mucosal Stains: Kaposi sarcoma can affect mucous membranes such as inside the mouth, nose, throat or anus. For this reason, purplish spots or swellings may also appear in the mouth or genital area.

Lymph Node Swelling: Kaposi's sarcoma can sometimes cause the lymph nodes to swell. These swellings are usually painless.

Internal Organ Involvement: Kaposi sarcoma can sometimes lead to tumor formation in internal organs. In this case, people may experience symptoms, and these symptoms may vary depending on the organs. For example, if there is involvement of the stomach or intestines, symptoms such as stomach pain, weight loss, or bleeding may occur.

Respiratory Problems: If Kaposi's sarcoma spreads to the lungs, symptoms such as breathing difficulties and shortness of breath may occur.

Symptoms of Kaposi sarcoma may vary depending on the patient's immune system status and the extent and location of the sarcoma. Therefore, it is important to see a doctor for a diagnosis of Kaposi sarcoma. It is important to remember that this cancer is often associated with conditions that weaken the immune system, such as HIV infection. Early diagnosis and treatment can help control the disease.

HOW IS IT DIAGNOSED?

Kaposi sarcoma is diagnosed using a number of clinical and laboratory methods. The first step is a physical examination of lesions on the skin and mucous membranes. Then, tissue samples are taken from the lesions through a process called biopsy and this sample is examined in the pathology laboratory. Pathology examination helps diagnose the presence of cancer cells and characteristic features specific to Kaposi sarcoma. Additionally, additional tests such as imaging studies, blood tests, and sometimes lymph node biopsies may often be required to confirm the diagnosis of Kaposi sarcoma and evaluate the spread of the disease. Diagnosis may vary depending on the patient's immune system status, the spread of the cancer, and the person's general health condition. Therefore, it is important to see a dermatology or oncologist for a diagnosis of Kaposi sarcoma.

WHAT ARE THE PATHOLOGICAL TYPES?

Pathological types of Kaposi sarcoma may be:

Classic Type: This type of Kaposi sarcoma usually occurs in older men and individuals with an intact immune system. Skin lesions usually grow slowly and are usually painless.

Epidemic Type: This type of Kaposi sarcoma is more common in people with HIV (Human Immunodeficiency Virus). This type of Kaposi's sarcoma usually spreads more quickly and can affect other organs.

Immunosuppressive Related Type: It is a type of Kaposi sarcoma that can be seen after organ transplantation or in people using immunosuppressive drugs. It may develop because the immune system is suppressed.

Endemic Type: The endemic type of Kaposi sarcoma is more common in Central and East Africa and usually occurs in young men. This type can also occur without being associated with HIV.

These pathological types reflect that Kaposi sarcoma may have different clinical features and prognoses. Each type may differ depending on immune system status and the extent of spread of the disease. Treatment and monitoring strategies may also vary depending on these factors. Diagnosis and treatment of Kaposi sarcoma is usually guided by an oncologist or dermatologist.

Image 2: HHV-8 virus may play a role in the development of Kaposi's sarcoma.

HOW IS TUMOR STAGING DONE?

Kaposi sarcoma tumor staging is a system used to evaluate the extent of cancer and the areas it affects. Staging of Kaposi sarcoma is usually based on the following factors:

Location and Spread of Tumor (T): The location of the tumor in the body and how far it has spread are evaluated. Tumors in different areas such as skin, mouth, internal organs and lymph nodes are evaluated in different ways.

Immune System Status (I): The patient's immune system status is an important factor in the staging of KS, especially in cases of HIV-associated KS. Immune system parameters such as CD4+ cell count are evaluated.

Presence of Systemic Disease (S): Systemic disease refers to diseases that affect the general condition of the body. This means that any other diseases (e.g. infections, kidney disease) as well as cancer are also taken into account.

Other Clinical Findings: This includes the patient's overall health, the severity of their symptoms, and any other related health issues.

The staging system is commonly used for AIDS-related KS and is generally classified as follows:

Early Stage (Good Prognosis): Limited skin lesions and lymph node involvement indicate a good immune system status and absence of systemic disease.

Advanced Stage (Poor Prognosis): It means extensive skin involvement, involvement of internal organs, weak immune system and/or the presence of serious systemic disease.

Kaposi sarcoma tumor staging helps evaluate the spread and severity of the cancer and helps determine the treatment approach. Staging is done based on data such as physical examination, imaging studies (e.g., x-rays, CT scans), and biopsy results. Treatment options and prognosis may vary depending on the patient's stage. Therefore, tumor staging is an important step for people diagnosed with Kaposi sarcoma and is performed by an oncologist.

HOW IS TREATMENT DONE ACCORDING TO STAGES?

Treatment of Kaposi sarcoma (KS) is determined according to the patient's risk status, health status, spread of the disease and the state of the immune system. The following approaches are generally used in the treatment of KS, depending on the risk:

Low Risk (Local or Limited Disease):

Local Treatments: Local treatments can be applied for limited skin lesions. These treatments may be cryotherapy (freezing treatment), topical treatments (medications applied to the skin), radiotherapy or surgical excision (removal of lesions).

Systemic Treatment: If immune system weakness is the primary cause of KS (for example, HIV-associated KS), antiretroviral therapy (ART) may help reduce KS lesions.

High Risk (Common or Advanced Stage Disease):

Systemic Chemotherapy: Systemic chemotherapy may be preferred in patients with widespread skin lesions, lymph nodes or internal organ involvement.

Immunotherapy and Targeted Therapies: In some cases, immunotherapeutic agents or treatments directed at specific molecular targets may be used.

Immune System Supporting Therapies: In HIV-associated KS, antiretroviral therapy (ART) can help control KS by strengthening the immune system.

Exceptions:

Organ Transplant Recipients: Reducing or changing immunosuppressive medications may be effective in treating KS in organ transplant recipients.

Advanced Age and Additional Diseases: For elderly patients or those with other serious diseases, treatment is customized taking into account their general health status and management of other diseases.

Each patient's situation is different and the treatment plan should be prepared individually. Treatment options and approaches depend on many factors, including the patient's general health, the stage of the disease, and the state of the immune system. Therefore, it is important to cooperate with oncology and infectious disease specialists and perform regular follow-up for the correct treatment approach.

Image 3: The incidence of Kaposi sarcoma is increased especially in individuals with HIV/AIDS.

WHAT ARE THE SYSTEMIC DRUGS USED IN TREATMENT?

Chemotherapy, hormonal drugs, smart drug treatments and immunotherapies used in the treatment of Kaposi sarcoma are listed below:

Chemotherapy drugs:

Paclitaxel: It is a chemotherapy drug frequently used in the treatment of Kaposi sarcoma. It can be applied systemically (spread throughout the body) or locally (regionally).

Liposomal Doxorubicin: It is another chemotherapy drug and is commonly used in the treatment of HIV-associated Kaposi sarcoma. Liposomal form has lower toxicity.

Smart Drug Therapies (Targeted Therapies):

Pazopanib: It is a targeted drug used in the treatment of Kaposi's sarcoma. It targets Vascular Endothelial Growth Factor (VEGF) receptors and works to control the growth of cancer cells by inhibiting blood vessel formation.

Sunitinib: It is another targeted drug used in the treatment of Kaposi sarcoma. It suppresses the growth of cancer cells by targeting VEGF receptors and other targets.

Antiretroviral Drugs: Treatment of HIV infection in patients with HIV-associated Kaposi sarcoma may help strengthen the immune system and fight Kaposi sarcoma.

Immunomodulator Drugs: Immunomodulatory drugs can help regulate the immune system and are sometimes used to treat Kaposi's sarcoma. These may include interferon alpha.

Treatment options may vary depending on the patient's overall health, the stage of Kaposi's sarcoma, and other individual factors. Treatment for Kaposi sarcoma is customized by an oncologist or specialist physician and tailored to the patient's needs.

HOW SHOULD FOLLOW-UP BE DONE AFTER RECOVERY?

Follow-up after Kaposi sarcoma treatment is important to assess the patient's general health status and the risk of Kaposi sarcoma recurrence. Tracking can be done in the following ways:

Routine Doctor Examination: Patients should be examined by an oncologist or specialist physician at regular intervals after treatment. These examinations are important to monitor for signs of recurrence of Kaposi sarcoma and to diagnose it early.

Imaging Tests: After treatment, you may be monitored to determine the spread or recurrence of the cancer, using imaging tests (e.g., x-ray, CT scan) if necessary.

Blood Tests: Blood tests may be done to evaluate a patient's immune system health and to watch for HIV or other immune system problems.

Antiretroviral Therapy: In case of HIV-associated Kaposi sarcoma, antiretroviral therapy should be continued. It may help maintain immune system health and reduce the risk of cancer recurrence.

Supportive Care: After treatment, supportive care is important to improve patients' quality of life and help them cope with post-treatment effects. Supportive care may include nutrition, pain control, psychological support, and other issues.

Follow-up after Kaposi sarcoma treatment is customized based on the patient's individual needs and type of treatment. Regular doctor's examinations and follow-up tests are vital to maintaining the patient's health and detecting early the risk of recurrence or progression of Kaposi's sarcoma.