Kaposi's sarcoma in childhood: an analysis of 100 cases from Uganda and relationship to HIV infection.

We report 100 cases of Kaposi's sarcoma (KS) in children under 15 years of age treated at the Uganda Cancer Institute in the 6-year period 1989-1994. The incidence of childhood KS has risen more than 40-fold in the era of AIDS, and 78% of 63 cases tested were seropositive for HIV-1. There were 63 boys and 37 girls. The median age was 4 years and the median age of onset was 33 months. Tumour distribution was lymphadenopathic and muco-cutaneous, with 2 major patterns: pattern I, oro-facial dominant (79%); and pattern II, inguinal-genital dominant (13%). A newly described herpes-like virus is implicated as the cause of KS (KSHV), and DNA sequences of this virus were present in all of 8 childhood cases tested. If KSHV is a direct cause of KS, this tumour distribution in children suggests mucosal routes of virus entry, possibly during birth or breast feeding. The dramatic increase of childhood KS implies that the prevalence of causative factors is rising in Uganda.