A case of Kaposi sarcoma (KS) with cutaneous and splenic involvement is reported. Carcinoma of the left colon and basal cell epithelioma arose eleven years after KS. Genetic, viral, and immunologic factors which may promote initiation and development of Kaposi sarcoma are reviewed. The responsibility of immunodeficiency, whether resulting from therapy or from other causes, in carcinogenesis is discussed. Prolonged survival may be seen after KS. Another primary malignant disease (lymphoma or solid tumor) may arise. The authors suggest that the risk of immunologic disorders, occurring spontaneously or induced by therapy, should be considered specifically for each patient with KS.

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