Smooth muscle tumors are now recognized as the second most frequent maligna
ncy in HIV infected children (1). The annual incidence of soft tissue tumor
s in the entire pediatric population is approximately 8.4 cases per million
children (2, 3). Of these at least 12 cases of leiomyomas and leiomyosarco
mas have been reported in children with the acquired immune deficiency synd
rome (AIDS) (4-11). This brief communication is the first report describing
the endoscopic appearance of these tumors in the gastrointestinal tract in
children. The clinical synopsis of the case is followed by a short review
of pediatric intestinal leiomyosarcomas, emphasizing the published literatu
re on children with AIDS, and the uncertainties regarding the histopatholog
ic diagnosis of these lesions.