Vol.:(0123456789) 1 3 Eur J Orthop Surg Traumatol DOI 10.1007/s00590-017-2019-4 ORIGINAL ARTICLE • TUMORS - PAEDIATRICS Pediatric soft tissue sarcoma of the limbs: clinical outcome of 97 patients Andrea Sambri 1  · Giuseppe Bianchi 1  · Ilaria Cucurnia 1  · Marco Gambarotti 1  · Davide Maria Donati 1   Received: 27 May 2017 / Accepted: 19 July 2017 © Springer-Verlag France SAS 2017 Keywords Pediatric · Childhood · Soft tissue sarcoma · Limbs · Extremities Introduction Soft tissue sarcomas (STS) represent a heterogeneous group of mesenchymal malignancies that account for about 8% of all neoplasms in childhood and adolescence. Approximately 50–60% of STS are rhabdomyosarcoma (RMS), while the remainder forms varied group of the so-called non-rhabdo- myosarcoma soft tissue sarcomas (NRSTS). Rhabdomyosarcoma is the most common STS in chil- dren younger than 14 years, and it is further divided into 3 histologic subtypes (embryonal, alveolar, pleomorphic). The most common sites of primary RMS are the head, the genitourinary tract and the limbs [1, 2]. In particular, those RMS afecting limbs and girdles are more likely to display alveolar histology and metastatic spread [3]. Non-rhabdomyosarcoma soft tissue sarcomas are a het- erogeneous group of adult-type STS encompassing more than 50 diferent histologic diagnoses arising from primitive mesenchymal tissue, which occur in children and adoles- cents [4–7]. They generally afect older children, increas- ing in incidence throughout adolescent years [8]. More fre- quently NRSTS arise in the extremities (limbs and girdles), although they can develop anywhere in the body, and are characterized by local aggressiveness and a propensity to metastasize that is correlated with their grade of malig- nancy [4, 9, 10]. The prognosis of pediatric STS has improved signifcantly during the past three decades thanks to a multidisciplinary therapeutic approach [11–13]. Surgery provides the best choice of local control of small resectable tumors in order to obtain margins free of tumor Abstract Purpose Soft tissue sarcomas (STS) of the extremities in children are a heterogeneous group of tumors with a very diferent prognosis for which optimal treatment remains controversial. Patients and methods We retrospectively evaluated 97 patients younger than 15 years old afected 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 classifcation. Over- all 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 afected by extremities RMS were confrmed to have the worst prognosis, in particular in case of metastasis at presentation. Diferently from adult patients, hand and feet locations are frequent site for STS and 2 cm diameter should be taken as cut of for higher risk of LR. Similarly to adulthood STS, grading correlates with prog- nosis in NRSTS. The identifcation of prognostic variables should enable risk-adapted therapies to be planned. * Andrea Sambri andrea_sambri@libero.it 1 Istituto Ortopedico Rizzoli, Bologna, Italy