Kaposi's sarcoma, multiple haemorrhagic sarcoma | |
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Characteristic purple lesions of Kaposi's sarcoma on the nose of an HIV-positive female.[1] | |
Pronunciation | |
Specialty | Oncology |
Symptoms | Purple colored skin lesions[4] |
Types | Classic, endemic, immunosuppression therapy-related, epidemic[4][5] |
Risk factors | Human herpesvirus 8 (HHV8), poor immune function[4][6] |
Diagnostic method | Tissue biopsy, medical imaging[4][6] |
Differential diagnosis | Blue rubber bleb nevus syndrome, pyogenic granuloma, melanocytic nevi, melanoma[6] |
Treatment | Surgery, chemotherapy, radiation therapy, biologic therapy[4] |
Frequency | 42,000 (new cases, 2018)[7] |
Deaths | 20,000 (2018)[7] |
Kaposi's sarcoma (KS) is a type of cancer that can form masses on the skin, in lymph nodes, in the mouth, or in other organs.[4][6] The skin lesions are usually painless, purple and may be flat or raised.[6][8] Lesions can occur singly, multiply in a limited area, or may be widespread.[6] Depending on the sub-type of disease and level of immune suppression, KS may worsen either gradually or quickly.[6] 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)).[8]
Classic, endemic, immunosuppression therapy-related (also known as iatrogenic), and epidemic (also known as AIDS-related) sub-types are all described.[8] 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.[8] Endemic KS is most common in Sub-Saharan Africa and is more aggressive in children, while older adults present similarly to classic KS.[8] Immunosuppression therapy-related KS generally occurs in people following organ transplantation and mostly affects the skin.[8] Epidemic KS occurs in people with AIDS and many parts of the body can be affected.[8] KS is diagnosed by tissue biopsy, while the extent of disease may be determined by medical imaging.[4][6][8]
Treatment is based on the sub-type, whether the condition is localized or widespread, and the person's immune function.[6] Localized skin lesions may be treated by surgery, injections of chemotherapy into the lesion, or radiation therapy.[6] Widespread disease may be treated with chemotherapy or biologic therapy.[4][6] In those with HIV/AIDS, highly active antiretroviral therapy (HAART) prevents and often treats KS.[8][9] In certain cases the addition of chemotherapy may be required.[9] With widespread disease, death may occur.[6]
The condition is relatively common in people with HIV/AIDS and following organ transplant.[6][8][9] Over 35% of people with AIDS may be affected.[10] KS was first described by Moritz Kaposi in 1872.[11][12] It became more widely known as one of the AIDS-defining illnesses in the 1980s.[11] KSHV was discovered as a causative agent in 1994.[11][13]