Kaposi Sarcoma

What is Kaposi Sarcoma?

Kaposi Sarcoma (KS) is a type of soft tissue sarcoma, which is a cancer that originates in the tissues that support and connect the body, such as muscles, tendons, fat, and blood vessels. Kaposi Sarcoma is caused by a virus known as Human Herpes Virus 8 (HHV-8). However, it is important to note that not everyone infected with HHV-8 will develop Kaposi Sarcoma. This type of cancer usually affects the skin, but it can also occur in the mucosal linings of the mouth, nose, throat, and anus.

Types of Kaposi Sarcoma

All types of Kaposi Sarcoma are caused by HHV-8, but they differ mainly in how and where the disease occurs. These types are often associated with the immune system’s ability to function properly:

1. Classic Kaposi Sarcoma:

• This form typically affects older men, particularly of Mediterranean, Eastern European, and Middle Eastern descent.

• Lesions usually develop slowly on the lower extremities (legs, ankles, feet). It is more common in men than in women, and the lesions may take 10 to 15 years to fully develop.

2. Epidemic (AIDS-related) Kaposi Sarcoma:

• This is the most common type of Kaposi Sarcoma and is associated with people who are HIV-positive or have Acquired Immunodeficiency Syndrome (AIDS).

• The cancer often spreads throughout the body, affecting not just the skin but also internal organs such as the lymph nodes, liver, spleen, lungs, and digestive tract.

3. Transplant-Associated (Immunosuppression-Related) Kaposi Sarcoma:

• This type occurs in people who have had organ transplants and are on immunosuppressive medications to prevent rejection of the new organ.

• The suppression of the immune system increases the risk of developing Kaposi Sarcoma, particularly in the skin, and sometimes in deeper tissues or organs.

4. Endemic (African) Kaposi Sarcoma:

• This type occurs in parts of Africa, even before the AIDS epidemic. It is more aggressive than the classic type and can affect younger people, sometimes even children.

Statistics

Globally, it is estimated that in 2020, about 34,270 people were diagnosed with Kaposi Sarcoma, and about 15,086 people died due to this disease. In the United States, the 5-year survival rate is around 75%. This rate can vary depending on whether the cancer has spread to other parts of the body and how well it responds to treatment.

Risk Factors for Kaposi Sarcoma

Several factors can increase the risk of developing Kaposi Sarcoma, including:

1. HHV-8 Infection:

• Kaposi Sarcoma is primarily caused by infection with Human Herpesvirus 8 (HHV-8). However, not everyone with HHV-8 develops Kaposi Sarcoma; the virus must interact with other risk factors to trigger the disease.

2. Immune Suppression:

• People with weakened immune systems, such as those with HIV/AIDS or individuals taking immunosuppressive drugs after organ transplants, are at higher risk.

3. Gender:

• Men are more likely to develop Kaposi Sarcoma than women.

4. Sexual Activity:

• Unsafe sexual practices that increase the risk of HIV or HHV-8 transmission can raise the likelihood of developing Kaposi Sarcoma.

Symptoms of Kaposi Sarcoma

The symptoms of Kaposi Sarcoma can vary, but some of the most common signs include:

1. Skin Lesions:

• Raised, discolored patches on the skin that can appear pink, red, purple, or dark brown. These lesions are often seen on the legs, face, or genitals.

2. Swelling (Lymphedema):

• Swelling in the arms, legs, or other body parts, caused by blockage in the lymphatic system.

3. Respiratory Symptoms:

• Persistent cough or chest pain if the cancer affects the lungs.

4. Digestive Symptoms:

• Abdominal pain, nausea, vomiting, or gastrointestinal bleeding if the cancer affects the digestive tract.

Diagnosis of Kaposi Sarcoma

To diagnose Kaposi Sarcoma, several tests may be conducted:

1. Biopsy:

• A sample of the affected tissue is removed and examined under a microscope to confirm the presence of cancer cells.

2. X-rays:

• Used to detect any spread of the cancer, particularly in the lungs.

3. CT Scan:

• A detailed imaging technique used to determine if the cancer has spread to other organs.

4. Endoscopy:

• A camera is inserted into the gastrointestinal tract or lungs to examine for lesions.

5. Photography:

• Lesions on the skin are photographed to monitor changes over time.

Treatment Options for Kaposi Sarcoma

1. Antiretroviral Therapy (ART):

• For people with HIV/AIDS, controlling the HIV virus with ART is often the first line of defense. ART alone can sometimes help shrink Kaposi Sarcoma lesions.

2. Surgery:

• There are two main types of surgery:

• Curettage and Electrodesiccation: Cancerous tissue is scraped off, and electrical current is applied to kill remaining cancer cells.

• Cryosurgery: Liquid nitrogen is used to freeze and destroy cancerous tissue.

3. Photodynamic Therapy:

• A light-sensitive drug is injected into the bloodstream and absorbed by cancer cells. A laser light is then applied to the area, activating the drug and destroying the cancer cells.

4. Radiation Therapy:

• High-energy rays are used to kill cancer cells. This is often used for localized lesions or to relieve symptoms.

5. Chemotherapy:

• Chemotherapy drugs, such as liposomal doxorubicin or paclitaxel, can be used to kill cancer cells. These drugs can have side effects like fatigue, nausea, vomiting, hair loss, and more.

6. Targeted Therapy:

• This treatment uses drugs like Pomalidomide (Pomalyst) to specifically target and kill cancer cells without harming normal cells.

7. Topical Medications:

• Creams like Imiquimod or Alitretinoin can be applied to the skin to shrink or stop the growth of lesions.

8. Immunotherapy:

• Drugs that boost the body’s immune system to fight cancer, such as Pembrolizumab or Nivolumab, may be used in advanced cases.

Follow-Up Care

After treatment, regular follow-up is essential. Patients will need ongoing blood tests, imaging scans, and physical examinations to monitor for recurrence or manage side effects. The follow-up schedule may vary based on individual health conditions and the extent of the disease.
Sarcoma is a complex disease that primarily affects individuals with compromised immune systems. Advances in HIV treatment and the use of targeted therapies have improved survival rates for people with this cancer. However, early detection and consistent medical care are crucial for managing this disease effectively.

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