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Kaposi Sarcoma: Causes, Symptoms & Treatment

Kaposi sarcoma is a rare vascular tumor caused by the herpesvirus HHV-8. It most commonly affects individuals with a weakened immune system.

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Things worth knowing about "Kaposi sarcoma"

Kaposi sarcoma is a rare vascular tumor caused by the herpesvirus HHV-8. It most commonly affects individuals with a weakened immune system.

What Is Kaposi Sarcoma?

Kaposi sarcoma (KS) is a malignant tumor originating from the endothelial cells lining blood and lymphatic vessels. It is caused by Human Herpesvirus 8 (HHV-8), also known as Kaposi sarcoma-associated herpesvirus (KSHV). The tumor typically presents as purple, reddish-brown, or bluish skin lesions, but can also affect internal organs. It was first described in 1872 by the Hungarian dermatologist Moritz Kaposi.

Types of Kaposi Sarcoma

There are four main clinical subtypes:

Classic Kaposi sarcoma: Predominantly affects older men of Mediterranean or Eastern European descent. It usually follows a slow course and mainly affects the lower limbs.
Endemic (African) Kaposi sarcoma: Found in certain regions of sub-Saharan Africa, can affect children, and may follow a more aggressive course.
Iatrogenic Kaposi sarcoma: Develops as a consequence of immunosuppressive therapy, such as after organ transplantation.
Epidemic (AIDS-associated) Kaposi sarcoma: The most common form in Western countries. Occurs in HIV-infected individuals with severely compromised immune systems and can be life-threatening.

Causes and Risk Factors

Infection with HHV-8 is a necessary but not sufficient cause of Kaposi sarcoma. Not every infected person develops the disease. The interaction between the virus and a weakened immune system plays a decisive role. Key risk factors include:

HIV infection or AIDS
Immunosuppressive medications (e.g., after organ transplantation)
Advanced age
Male sex
Origin from endemic regions (e.g., sub-Saharan Africa, Mediterranean countries)

Symptoms

Symptoms of Kaposi sarcoma depend on the affected body region:

Skin: Painless purple, reddish, or brown spots, nodules, or plaques, often on the feet, legs, or face
Mucous membranes: Lesions in the mouth, throat, or on the conjunctiva
Gastrointestinal tract: Abdominal pain, bloating, diarrhea, occasionally bleeding
Lungs: Cough, shortness of breath, coughing up blood
Lymph nodes: Swelling and edema

Diagnosis

Diagnosis of Kaposi sarcoma involves several steps:

Clinical examination: Assessment of characteristic skin lesions by a physician
Skin biopsy: Removal of a tissue sample for histological examination – this is the gold standard for diagnosis
Endoscopy: When gastrointestinal involvement is suspected
Imaging: X-ray, CT, or MRI to assess organ involvement
HIV testing and immune status: Measurement of CD4 cell count and HIV viral load when AIDS-associated KS is suspected

Treatment

Treatment depends on the type of Kaposi sarcoma, the extent of the disease, and the overall health of the patient:

Local Therapies

Surgical excision of small, isolated lesions
Cryotherapy (freezing of lesions)
Radiation therapy for locally confined tumors
Intralesional chemotherapy (e.g., vinblastine injected directly into the lesion)

Systemic Therapies

Antiretroviral therapy (ART): In HIV-associated KS, successful ART often leads to significant regression of lesions by restoring immune function.
Systemic chemotherapy: Liposomal doxorubicin or paclitaxel for advanced or systemic disease
Reduction of immunosuppression: In iatrogenic cases, where medically feasible
Immunotherapy and targeted therapies: Currently under clinical investigation

Prognosis

Prognosis depends strongly on the underlying cause and the extent of the disease. Classic KS often remains stable over many years. The prognosis of AIDS-associated KS has improved significantly with modern antiretroviral therapy. Organ involvement, particularly of the lungs, substantially worsens the prognosis.

References

Krown SE et al. - Kaposi Sarcoma in a Changing Treatment Landscape. The Oncologist, 2011.
Dezube BJ - Clinical presentation and natural history of AIDS-related Kaposi's sarcoma. Hematology/Oncology Clinics of North America, 1996.
World Health Organization (WHO) - Kaposi Sarcoma. WHO Cancer Fact Sheets, 2023. Available at: https://www.who.int

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