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: P C McGrath, J P Neifeld, S Kay, A M Salzberg
• Journal: Journal of pediatric surgery (J Pediatr Surg) Vol. 23 Issue 10 Pg. 939-41 (Oct 1988) ISSN: 0022-3468 [Print] United States
• PMID: 3069997 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Child
• Child, Preschool
• Colonic Neoplasms (surgery)
• Female
• Humans
• Infant
• Infant, Newborn
• Intestinal Neoplasms (surgery)
• Jejunal Neoplasms (surgery)
• Leiomyosarcoma (surgery)
• Male