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Leiomyosarcoma in childhood and adolescence
E S Hwang1, W Gerald, N Wollner
1Department of Surgery, Memorial-Sloan Kettering Cancer Center, New York, New York 10021, USA.
Annals of Surgical Oncology
|April 1, 1997
Summary
Pediatric leiomyosarcoma shows good initial survival, but long-term outcomes progressively worsen. Tumor grade correlates with surgical margin success, impacting prognosis in young patients.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Cancer Research
Background:
- Leiomyosarcoma in patients under 21 years old is rare, with limited reported case series.
- This study reviews institutional experience to understand disease characteristics, relapse patterns, and outcomes in pediatric leiomyosarcoma.
Purpose of the Study:
- To characterize leiomyosarcoma in patients younger than 21 years.
- To identify prognostic factors and survival patterns in this pediatric population.
Main Methods:
- Retrospective review of 21 patients diagnosed with leiomyosarcoma at the institution.
- Survival analysis using the Kaplan-Meier method to estimate overall survival.
Main Results:
- Ninety-five percent of patients underwent initial wide local excision; complete resection with negative margins was achieved in 48%.
- High-grade tumors were associated with incomplete resection and a strong correlation between tumor grade and surgical margins.
- The median survival was 9.3 years, with a 5-year survival rate of 79% and a 10-year rate of 49%, indicating a progressive decline in survival over time.
Conclusions:
- Pediatric leiomyosarcomas have a favorable initial survival rate that diminishes over time.
- Prognostic factors observed in adult leiomyosarcoma series appear consistent but require larger pediatric cohorts for definitive validation.
- Tumor grade is a significant factor, correlating with surgical margin status in pediatric leiomyosarcoma.