Pediatric soft tissue sarcoma of the limbs: clinical outcome of 97 patients

Purpose: Soft tissue sarcomas (STS) of the extremities in children are a heterogeneous group of tumors with a very different prognosis for which optimal treatment remains controversial. Patients and methods: We retrospectively evaluated 97 patients younger than 15 years old affected by limb soft tissue sarcomas. All cases were histologically revised, and tumor grade was assessed according to the FNLCC system. Thirty-two were rhabdomyosarcoma (RMS) and 65 non-rhabdomyosarcoma (NRMSTS); among these, 40 (61.5%) were grade 3 according to FNLCC classification. Overall survival, local recurrence and distant metastasis were analyzed. Results: Overall survival was 77.8% at 5 years and 69.7% at 10 years. Among grade 3 tumors, RMS had a worse prognosis over NRSTS. Similarly, tumors larger than 5 cm had a worse prognosis compared to smaller ones. Local recurrence-free survival was 90.7% at 5 years and 87.1% at 10 years with a better local control in grade 3 NRSTS over RMS and in tumors smaller than 2 cm. Conclusion: Children affected by extremities RMS were confirmed to have the worst prognosis, in particular in case of metastasis at presentation. Differently from adult patients, hand and feet locations are frequent site for STS and 2 cm diameter should be taken as cut off for higher risk of LR. Similarly to adulthood STS, grading correlates with prognosis in NRSTS. The identification of prognostic variables should enable risk-adapted therapies to be planned.