What is Kaposi's sarcoma?

From the Guidelines

Kaposi sarcoma is a type of cancer that develops from the cells that line lymph or blood vessels, causing purple, red, or brown patches, plaques, or tumors on the skin and sometimes affecting internal organs, and it is caused by human herpesvirus 8 (HHV-8), also known as Kaposi sarcoma-associated herpesvirus (KSHV) 1.

Types of Kaposi Sarcoma

There are four main types of Kaposi sarcoma:

• Classic (affecting older men of Mediterranean or Eastern European descent)
• Endemic (found in parts of Africa)
• Iatrogenic (occurring in people on immunosuppressive medications)
• Epidemic or AIDS-related (the most common type in the United States, affecting people with weakened immune systems due to HIV) 2, 3, 4 ### Treatment of Kaposi Sarcoma Treatment depends on the type and extent of disease but may include:
• Antiretroviral therapy for HIV-positive patients
• Local treatments like cryotherapy or radiation for limited skin lesions
• Systemic chemotherapy for widespread disease
• Medications like liposomal doxorubicin or paclitaxel are commonly used chemotherapy agents 1, 4 ### Disease Progression The disease's progression varies widely - some cases grow slowly over years while others progress rapidly, especially in people with severely compromised immune systems 3 ### Management Management of AIDS-related Kaposi sarcoma depends on location and extent of disease, and patients with limited cutaneous disease that is asymptomatic and cosmetically acceptable to the patient may be treated with ART alone, while those with symptomatic and/or cosmetically unacceptable limited cutaneous disease should be treated with ART and with therapy that is minimally invasive with the least toxicity possible 2, 3

From the Research

Definition and Characteristics of Kaposi Sarcoma

• Kaposi's sarcoma (KS) is a multicentric angioproliferative cancer of endothelial cells (ECs) caused by Human Herpesvirus 8 (HHV8) 5.
• It is characterized by clinical heterogeneity depending on the host immune conditions.
• KS can present in different forms, including classic KS, endemic KS, and iatrogenic KS, and is often associated with HIV infection 6.

Clinical Presentation and Diagnosis

• The clinical presentation of KS varies from an indolent slowly progressive behavior, generally limited to the skin, to an aggressive and rapidly progressing disease 5.
• In more than 50% of cases, the skin lesions are often associated with a more or less important visceral involvement, particularly to the oral cavity and the gastrointestinal tract 5.
• Diagnosis is typically based on clinical presentation and confirmed by biopsy.

Treatment Options

• Treatment for KS depends on the stage and severity of the disease, as well as the patient's overall health and immune status.
• For patients with HIV-associated KS, antiretroviral therapy (ART) is often the first line of treatment, and can lead to significant improvement or complete regression of the disease 5, 6.
• Chemotherapy, such as pegylated liposomal doxorubicin (PLD) or paclitaxel, may be used in combination with ART for more advanced or aggressive disease 7, 8, 9.
• Local treatments, such as cryotherapy or radiotherapy, may also be used for limited skin involvement 5.

Prognosis and Outcomes

• The prognosis for KS varies depending on the stage and severity of the disease, as well as the patient's overall health and immune status.
• With effective treatment, including ART and chemotherapy, many patients with KS can experience significant improvement or complete regression of the disease 5, 9.
• However, KS remains a significant cause of morbidity and mortality, particularly in resource-limited settings or in patients with advanced or untreated HIV infection 7, 6.