Kaposi Sarcoma: Symptoms, Causes, Treatment

What are the symptoms of Kaposi sarcoma?

Kaposi sarcoma is a type of cancer that causes the growth of abnormal cells in the skin and underlying tissues. The symptoms of Kaposi sarcoma can vary depending on the stage of the disease and the location of the lesions. Here are some common symptoms of Kaposi sarcoma:

Cutaneous Kaposi Sarcoma (most common form)

• Pink or red to purple-colored patches or nodules on the skin
• Lesions may be flat, raised, or nodular
• Lesions may be single or multiple
• May appear on any part of the body, but often on the lower extremities, face, and trunk
• May cause itching or pain

Advanced Stages

• Lesions may become more widespread and merge to form large areas of red or purple discoloration
• Lesions may break down and ooze fluid (ulcerate)
• Skin lesions may become thickened and hard (fibrotic)
• Lesions may cause pain, swelling, and stiffness

Other Symptoms

• Lymphedema (swelling) in the affected limb
• Pain in the affected limb or joint
• Fever
• Weight loss
• Night sweats
• Fatigue

Internal Organ Involvement (less common)

• Gastrointestinal Kaposi sarcoma:
  • Bleeding in the stomach, intestines, or rectum
  • Abdominal pain
  • Diarrhea or constipation
  • Weight loss
  • Fatigue
• Pulmonary Kaposi sarcoma:
  • Coughing up blood (hemoptysis)
  • Chest pain
  • Difficulty breathing (dyspnea)
• Other internal organs may be affected, such as the liver, kidneys, or brain

It’s essential to note that Kaposi sarcoma can also present with no symptoms at all, and diagnosis may be made incidentally during a routine medical examination or through imaging studies.

If you suspect you have Kaposi sarcoma or have concerns about any unusual skin changes, consult a healthcare professional for a proper evaluation and diagnosis.

What are the causes of Kaposi sarcoma?

Kaposi sarcoma is a type of cancer that is caused by the human herpesvirus 8 (HHV-8). This virus is a member of the herpesvirus family and is also known as Kaposi’s sarcoma-associated herpesvirus (KSHV).

Risk Factors

1. HIV/AIDS: Kaposi sarcoma is more common in people with HIV/AIDS, particularly those who have not received antiretroviral therapy (ART) or have low CD4 cell counts.
2. Weakened immune system: People with compromised immune systems, such as those with cancer, taking immunosuppressive drugs, or undergoing organ transplantation, are at higher risk of developing Kaposi sarcoma.
3. Age: Kaposi sarcoma is more common in older adults, especially those over 60 years old.
4. Genetic predisposition: Some people may be more susceptible to Kaposi sarcoma due to genetic factors.
5. Environmental factors: Exposure to certain environmental toxins or substances, such as pesticides or benzene, may increase the risk of developing Kaposi sarcoma.

Transmission

HHV-8 is primarily transmitted through:

1. Sexual contact: HHV-8 can be transmitted through sexual contact with an infected person.
2. Mother-to-child transmission: HHV-8 can be transmitted from an infected mother to her child during childbirth or breastfeeding.
3. Organ transplantation: HHV-8 can be transmitted through an organ transplant from an infected donor.

Other risk factors

1. Male gender: Men are more likely to develop Kaposi sarcoma than women.
2. African or Mediterranean descent: People of African or Mediterranean descent are at higher risk of developing Kaposi sarcoma due to higher rates of HHV-8 infection in these populations.
3. Previous skin conditions: Certain skin conditions, such as eczema or psoriasis, may increase the risk of developing Kaposi sarcoma.

It’s essential to note that most people with HHV-8 infection do not develop Kaposi sarcoma, and many people who develop Kaposi sarcoma have no known risk factors.

How is the diagnosis of Kaposi sarcoma made?

The diagnosis of Kaposi sarcoma (KS) is typically made through a combination of clinical evaluation, physical examination, and diagnostic tests. Here’s a step-by-step guide to the diagnostic process:

Clinical Evaluation

1. Medical history: The healthcare provider will ask questions about your medical history, including any previous skin conditions, cancers, or immunosuppressive therapy.
2. Physical examination: A thorough physical examination is performed to assess the extent and location of the skin lesions.

Diagnostic Tests

1. Biopsy: A biopsy is usually the most effective way to diagnose KS. A sample of the affected skin tissue is removed and sent to a laboratory for examination.
   • Punch biopsy: A small sample of skin tissue is removed using a specialized instrument called a punch biopsy.
   • Excisional biopsy: A larger sample of skin tissue is removed surgically.
2. Histopathology: The biopsy sample is examined under a microscope to look for characteristic features of KS, such as:
   • Abnormal growth of blood vessels
   • Infiltration of immune cells
   • Malignant cells
3. Imaging studies: Imaging tests may be performed to assess the extent of the disease and monitor treatment response:
   • X-rays: Chest X-rays to evaluate lung involvement
   • Computed Tomography (CT) scans: To evaluate internal organ involvement
   • Magnetic Resonance Imaging (MRI): To evaluate internal organ involvement and detect lesions in the brain or other organs
4. Serological tests: Blood tests may be performed to detect HHV-8 antibodies:
   • ELISA (Enzyme-Linked Immunosorbent Assay): Detects antibodies against HHV-8 in the blood
   • Western blot: Confirms the presence of HHV-8 antibodies
5. Lymph node evaluation: Lymph node involvement may be assessed through physical examination, imaging studies, or lymph node biopsy.

Staging

After diagnosis, the disease is staged based on the extent of involvement:

1. Stage I: Limited disease, with only skin lesions present.
2. Stage II: Disease has spread to lymph nodes or internal organs.
3. Stage III: Disease has spread to multiple internal organs.

A multidisciplinary team of healthcare providers, including dermatologists, oncologists, and radiologists, work together to diagnose and manage KS patients. Early detection and prompt treatment can improve outcomes for individuals with Kaposi sarcoma.

What is the treatment for Kaposi sarcoma?

The treatment for Kaposi sarcoma (KS) depends on the stage of the disease, the location of the lesions, and the patient’s overall health. Here are the common treatment options:

Surgical Treatment

1. Excision: Surgical removal of individual lesions or a specific area of affected skin.
2. Mohs surgery: A specialized surgical technique that involves removing thin layers of skin tissue and examining each layer under a microscope to ensure all cancer cells are removed.

Cytotoxic Chemotherapy

1. Single-agent chemotherapy: Chemotherapy drugs like doxorubicin (Adriamycin), vinblastine, and dacarbazine are commonly used to treat KS.
2. Combination chemotherapy: Combination regimens may include multiple chemotherapy drugs to enhance effectiveness.

Targeted Therapy

1. Bevacizumab: An antibody that targets vascular endothelial growth factor (VEGF), which is essential for tumor growth and survival.
2. Sorafenib: A multikinase inhibitor that targets VEGF and other kinases involved in tumor growth.

Immunotherapy

1. Interferon-alpha: An immunomodulatory agent that stimulates the immune system to fight cancer cells.
2. Interleukin-2 (IL-2): A cytokine that activates immune cells to target cancer cells.

Other Treatments

1. Radiation therapy: May be used to treat localized lesions or areas with extensive disease.
2. Palliative care: Symptomatic relief for pain, bleeding, or other symptoms associated with advanced disease.

Treatment Approaches for Specific Stages

1. Stage I and II: Excision or Mohs surgery may be sufficient for limited disease.
2. Stage III: Chemotherapy or targeted therapy may be added to surgical treatment.
3. Stage IV: Systemic chemotherapy, immunotherapy, or a combination of these approaches may be necessary.

Monitoring and Follow-up

Regular follow-up appointments with a healthcare provider are crucial to monitor the response to treatment, detect any recurrences, and adjust treatment as needed.

It’s essential to note that Kaposi sarcoma is a complex disease, and treatment plans may vary depending on individual circumstances. Your healthcare provider will develop a personalized treatment plan based on your specific needs and stage of disease.