Kaposi's sarcoma

Kaposi's sarcoma (KS) is a type of cancer that can form masses in the skin, in lymph nodes, in the mouth, or in other organs. The skin lesions are usually painless, purple and may be flat or raised. Lesions can occur singly, multiply in a limited area, or may be widespread. Depending on the sub-type of disease and level of immune suppression, KS may worsen either gradually or quickly. Except for Classical KS where there is generally no immune suppression, KS is caused by a combination of immune suppression (such as due to HIV/AIDS) and infection by Human herpesvirus 8 (HHV8 – also called KS-associated herpesvirus (KSHV)).

Kaposi's sarcoma, multiple haemorrhagic sarcoma
Other namesKaposi sarcoma
Kaposi sarcoma. Characteristic purple lesions on the nose in an HIV-positive female.
Pronunciation
  • /kæˈpsiz/, /ˈkɑːpəsiz, ˈkæpə-/
SpecialtyOncology
SymptomsPurple colored skin lesions
TypesClassic, endemic, immunosuppression therapy-related, epidemic
Risk factorsHuman herpesvirus 8 (HHV8), poor immune function
Diagnostic methodTissue biopsy, medical imaging
Differential diagnosisBlue rubber bleb nevus syndrome, pyogenic granuloma, melanocytic nevi, melanoma
TreatmentSurgery, chemotherapy, radiation therapy, biologic therapy
Frequency42,000 (new cases, 2018)
Deaths20,000 (2018)

Classic, endemic, immunosuppression therapy-related (also known as iatrogenic), and epidemic (also known as AIDS-related) sub-types are all described. Classic KS tends to affect older men in regions where KSHV is highly prevalent (Mediterranean, Eastern Europe, Middle East), is usually slow-growing, and most often affects only the legs. Endemic KS is most common in Sub-Saharan Africa and is more aggressive in children, while older adults present similarly to classic KS. Immunosuppression therapy-related KS generally occurs in people following organ transplantation and mostly affects the skin. Epidemic KS occurs in people with AIDS and many parts of the body can be affected. KS is diagnosed by tissue biopsy, while the extent of disease may be determined by medical imaging.

Treatment is based on the sub-type, whether the condition is localized or widespread, and the person's immune function. Localized skin lesions may be treated by surgery, injections of chemotherapy into the lesion, or radiation therapy. Widespread disease may be treated with chemotherapy or biologic therapy. In those with HIV/AIDS, highly active antiretroviral therapy (HAART) prevents and often treats KS. In certain cases the addition of chemotherapy may be required. With widespread disease, death may occur.

The condition is relatively common in people with HIV/AIDS and following organ transplant. Over 35% of people with AIDS may be affected. KS was first described by Moritz Kaposi in 1872. It became more widely known as one of the AIDS-defining illnesses in the 1980s. KSHV was discovered as a causative agent in 1994.

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