Kaposi Sarcoma Forms

Kaposi Sarcoma (KS) is an endothelial cell cancer with an inflammatory component, which has multiple heterogenous clinical presentations.

Classic (aka sporadic):

 - Presentation: Typically confined to lower limbs with few lesions. Visceral and mucosal disease rare and usually occurs in GI tract

 - Progression: Usually indolent; rarely aggressive and disseminated

Endemic:

 - Presentation: Children often present with multiple lymph nodes with lymphedema and very aggressive natural history of disease, including visceral dissemination. Adults present with lower-limb lesions that resemble classic KS

 - Progression: In children, progression is often aggressive with widespread lymphadenopathy and visceral involvement. In adults, progression is indolent or locally invasive but occasionally has visceral involvement

AIDS-related (aka epidemic):

 - Presentation: Multiple cutaneous lesions on the limbs, trunk, and face. Mucosal lesions are common (identified in 20% of patients) and visceral involvement is seen in 15% of patients. Patients can also present with tumor-associated edema

 - Progression: May follow an indolent course but visceral involvement is not uncommon and may be aggressive. It may regress with effective ART

Iatrogenic:

 - Presentation: Often presents as cutaneous KS lesions but both mucosal and rarely visceral disease can occur

 - Progression: Usually localized but may involve organs. It may regress with reduction in immunosuppression or with modification of immunosuppressive regimen

MSM without HIV infection:

 - Presentation: May occur at any skin sites, usually with few lesions. Visceral and mucosal disease is rare

 - Progression: Usually indolent, although disseminated disease has been



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