Aggressive Kaposi's sarcoma in Zambia, 1983.

From 1975 to 1982 between 8 and 12 new cases of Kaposi's sarcoma (KS) were seen each year in Lusaka, Zambia, and the clinical presentation and tumour behaviour conformed to descriptions of endemic KS from Uganda and Kenya. 23 patients presented with KS in 1983. 10 men, mean age 41, presented with typical symptoms and signs (nodules or plaques on oedematous limbs, with florid tumours or woody infiltration) and all 10 patients responded promptly to actinomycin D and vincristine. 13 patients (10 men and 3 women), mean age 27, presented with unusual symptoms and signs, including generalised symmetrical lymphadenopathy, oral or gastrointestinal lesions, respiratory distress, gross weight loss, and absence of nodules or plaques on the limbs. 8 of 13 patients with atypical KS failed to maintain an initial response to chemotherapy and died before the end of 1983, but there were no deaths amongst patients with endemic disease.