INFANTILE INTESTINAL LEIOMYOSARCOMA - SURGICAL RESECTION (WITHOUT ADJUVANT THERAPY) FOR CURE

A 7-week-old boy presented with a 6-week history of failure to thrive, acute intestinal obstruction, and an apparently irreducible intussusc eption (noted on contrast enema). He underwent abdominal exploration, during which a cecal mass was identified and resected. The mass proved to be a leiomyosarcoma. Histologically, it was an intermediate-grade malignancy with a predicted 5-year survival rate of 16% to 23% based o n data from the adult experience. Three years after resection and with out having received adjuvant therapy, he is healthy and free of diseas e. A review of the literature showed that in infants these tumors are predominantly colonic, compared with the predilection for small intest inal lesions found in the older pediatric and adult populations. Infan tile intestinal leiomyosarcomata are rare malignancies that do well if complete surgical excision of the disease can be accomplished. The hi stological prognostic indicators proposed for intestinal leiomyosarcom as in the adult population cannot be extrapolated to infants because w hen they occur in infants, they appear to be less aggressive, and thes e patients do well without adjuvant therapy. Copyright (C) 1996 by W.B . Saunders Company