Kaposi’s sarcoma (KS) is a multicentric angioproliferative cancer of endothelial origin that usually occurs in patients with immunodeficiency, such as Human Immunodeficiency Virus (HIV) or transplantation. KS-associated herpesvirus (KSHV), also known as human herpesvirus-8 (HHV-8), is associated with the development of KS. In the setting of HIV infection, the incidence of KS has dramatically decreased after the introduction of Highly Active Antiretroviral Therapy (HAART). HAART represents the first treatment step for slowly progressive disease, while chemotherapy (CT) plus HAART is indicated for visceral and/or rapidly progressive disease. Target therapies, based on anti-angiogenic agents as well as metalloproteinase and cytokine signaling pathway inhibitors, have been developed more recently and used for patients with progressive disease despite chemotherapy and/or HAART.

In this paper, we review the most recent data on KS epidemiology, pathogenesis and therapy.