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Principles in the management of pediatric intestinal leiomyosarcomas
P C McGrath1, J P Neifeld, S Kay
1Department of Surgery, Medical College of Virginia, Richmond 23298-0011.
Insights
Pediatric intestinal leiomyosarcomas are rare. These childhood tumors present differently and have a better prognosis than adult leiomyosarcomas, suggesting a distinct natural history.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Gastrointestinal Oncology
Background:
- Primary intestinal leiomyosarcomas in children are rare, with limited data on their clinical behavior.
- Understanding the natural history of these pediatric tumors is crucial for effective treatment strategies.
Observation:
- This report details two pediatric cases of jejunal leiomyosarcoma, adding to the 20 previously reported in the literature.
- Patients presented with symptoms such as intestinal obstruction or perforation.
- Tumor grade and resectability varied, influencing patient outcomes.
Findings:
- Complete resection was achieved in a higher proportion of pediatric cases compared to adults.
- Pediatric intestinal leiomyosarcomas demonstrated a more favorable prognosis than their adult counterparts.
- One patient with grade 1 tumor, completely resected, had no evidence of disease 14 years later.
Implications:
- The distinct clinical presentation and improved prognosis suggest pediatric intestinal leiomyosarcomas have a unique natural history compared to adults.
- Further research into pediatric-specific treatment protocols may improve outcomes for these rare tumors.
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.