Background and aims Paediatric soft tissue sarcomas are rare tumours that account for about 7% of all childhood cancers. 50–60% of these are rhabdomyosarcoma (RMS), while the remainder are non rhabdomyosarcoma soft tissue sarcomas (NRSTS). To evaluate the clinical characteristics, treatment modalities and outcome of paediatric soft tissue sarcomas.

Methods A retrospective analysis of data from 51 patients diagnosed and treated from 2003 to 2013.

Results 51 patients, 30 male and 21 female, median age of 10 years old at diagnosis. 92% showed a mass at diagnosis. The most common site of disease was the extremities (20 cases). Histology: 51% RMS (embryonal: 12 patients; alveolar: 12 patients; other: 2 patients) and 49% NRSTS (PNET 5 patients; malignant peripheral nerve sheath tumour: 3 patients; other: 17 patients). IRS group: 29% group I, 14% group II, 28% group III and 29% group IV. Most patients received multimodality therapy (radiotherapy, chemotherapy and surgery). 31% (16 patients) died due to disease progression: 7 alveolar RMS, 11 ≥ 10 years old and 13 group III-IV. Time from diagnosis to death was between 7 months and 5 years. Of the 34 living patients, 74% were in first complete remission. The median follow-up time was 38 months.

Conclusions Alveolar RMS is more common in the extremities and the embryonal in the genitourinary tract. NRSTS are more common in children ≥10 years. The absence of metastases and the tumour size ≤5 cm were associated with a better prognosis (p < 0.05).