Abstract
Background: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) represent about 3% of all childhood malignancies and about 50% of all soft tissue sarcomas. The heterogeneity of these tumors accounts for the paucity of information regarding their natural history and treatment guidelines. Therefore much of the evidence regarding the therapy has been extrapolated from adult studies and few pediatric case series. The purpose of this study is to evaluate our clinical experience with NRSTS in the pediatric population over the past 50 years.
Objectives: To evaluate the prognostic variables, event free survival and overall survival of childhood NRSTS.
Methods: We reviewed the medical records of all patients less than 21 years of age with NRSTS treated at our hospital between 1945 and 2001. Patients with Ewing's family of soft tissue sarcomas were excluded from the analysis. We collected the demographic, disease, histopathology and treatment data of 80 patients in whom all the data were available. The patient's age ranged from 1 month to 20 years (median 11.5 years). The median follow up time was 6 years (1 month to 46 years). Event free survival (EFS) and overall survival (OS) was calculated using Kaplan-Meier survival method.
Results: Of the 80 patients, 34 (42.5%) were able to achieve a complete surgical resection. Forty four (55%) received external beam radiotherapy and adjuvant chemotherapy was given to 57 (71.3%) cases. The EFS was significantly higher for patients with Stage I/II tumors (73%) as compared to Stage III/IV tumors (23%) [EFS, log rank = 18.8, p<0.001; OS, log rank = 21.9, p<0.001]. Of the different treatment modalities only complete surgical resection had a significant impact on survival (log rank = 8.9, p<0.003). Relative to other sites, chest and abdominal tumors had advanced stages (Chi Square = 22.9, p = 0.029). None of the other variables such as age, sex or race impacted survival in our study.
Conclusions: Our data show that surgical resection and tumor stage are the most important prognostic variables in childhood NRSTS. Wide surgical resection of the primary tumor with negative margins continues to be the mainstay of therapy. Radiotherapy and adjuvant chemotherapy should be reserved for patients with inadequate margins or those in whom a complete surgical resection can be facilitated by these modalities.
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Kalpatthi, R., Ruymann, F., Bayoumy, M. et al. 21 Prognostic Factors in Childhood Nonrhabdomyosarcoma Soft Tissue Sarcomas. Pediatr Res 58, 819 (2005). https://doi.org/10.1203/00006450-200510000-00051
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DOI: https://doi.org/10.1203/00006450-200510000-00051