Anaplastic Kaposi's sarcoma is a rare form of vascular tumor, clinically notable for its high local aggressiveness, propensity for deep invasion and increased metastatic capacity. This cancer arises in patients with a history of Kaposi's sarcoma (KS) and because of its rarity, few clinical data are available. To gain insight into this rare disease, we present eight cases of anaplastic KS which developed in eight out of 578 (1.4%) patients suffering from classic KS and followed in our department over a period of 30 years. Clinically, seven patients presented with deep tissue invasion. All the anaplastic cases underwent systemic chemotherapy and five received a non-conservative surgical therapy. Four (50%) out of the eight patients are now in complete remission, while three (37.5%) of them died of anaplastic Kaposi's sarcoma and one (12.5%) died of pneumonia after an excellent response to chemotherapy alone. Our findings suggest that in patients with anaplastic KS, non-conservative surgery associated with systemic chemotherapy may lead to complete remission, avoiding the poor outcome reported in previous cases.
Keywords: Kaposi's sarcoma; anaplastic; chemotherapy; lymphedema; prognosis; treatment.