Non–rhabdomyosarcoma soft-tissue sarcoma in children. 20 years experience (1996-2006) from a single institute

Background: Non – rhabdomyosarcoma soft – tissue sarcoma ( NMSTS ) include an assorted group of tumours accounting for 7% of all childhood malignancies and around 50% of all sarcomas in children. Material – Methods: A total of 25 patients with primary NRSTS were offered curative between January 1996 and June 2016. All patients had a comprehensive clinical evaluation and a biopsy performed for confirmation of the diagnosis. The median age was 5 years (1 month – 14 years ) with 16 males and 9 females. The predominant histological types were: synovial sarcoma (N: 4), exosteous Ewing sarcoma (N: 8), fibrosarcoma (N:4), liposarcoma (N: 1), clear cell sarcoma (N: 2), ASPS (N:1), DSRCT ( N:3 ), infantile HPC (N:1), HFSP ( N: 1). The most common site was the extremities (N: 15). Upfront surgery was performed in 5 patients ( 3 with fibrosarcoma, 1 with HPC, 1 with HFSP). The patients with Ewing sarcoma were treated according to Euro –Ewing 99 protocol. The others received chemotherapy (combination of vincristine, ifosfamide and doxorubicin) and post operative radiotherapy. Results: Median follow – up was 79 months (16 – 232). There was relapse in 6/25 ( 24 %) ( 2 with extra skeletal Ewing sarcoma, 1 with fibrosarcoma, 3 with DSRCT ). 19/25 are alive and disease free (OS: 76%, EFS 69%). Conclusion: NRSTS incorporates varied soft tissue sarcoma which is essential due the rarity of individual tumour subtypes. Surgery is the cornerstone of treatment however newer approaches are being evaluated for improving outcomes.