title: Principles in the management of pediatric intestinal leiomyosarcomas.
abstract: Primary intestinal leiomyosarcomas in children are unusual tumors with little known about their natural history. Two patients (aged 9 and 10 years) with leiomyosarcoma of the jejunum treated at our institution are reported and added to the 20 other patients reported in the literature. The first patient had a grade 1 leiomyosarcoma completely resected, received no additional therapy, and remains alive with no evidence of disease 14 years later. The second patient presented with a perforated grade 2 leiomyosarcoma which was incompletely resected; he received combination chemotherapy with an initial good response, but eventually died from sarcomatosis 7 years after initial diagnosis. The 22 cases of pediatric intestinal leiomyosarcoma presented more commonly with obstruction or perforation, were able to be completely resected more often, and appear to have a better prognosis than in adults. Thus, intestinal leiomyosarcomas in children appear to have a natural history different from that of the same tumor arising in adults.
authors.affiliation: Department of Surgery, Medical College of Virginia, Richmond 23298-0011.
authors.forename: P C
authors.initials: PC
authors.lastname: McGrath
authors.forename: J P
authors.initials: JP
authors.lastname: Neifeld
authors.forename: S
authors.initials: S
authors.lastname: Kay
authors.forename: A M
authors.initials: AM
authors.lastname: Salzberg
mesh_terms: D002648:Child; D002675:Child, Preschool; D003110:Colonic Neoplasms / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007414:Intestinal Neoplasms / Q000601:surgery*; D007580:Jejunal Neoplasms / Q000601:surgery; D007890:Leiomyosarcoma / Q000601:surgery*; D008297:Male