Pediatric non-rhabdomyosarcoma soft tissue sarcoma: heterogeneous group of rare tumors treated with a multidisciplinary approach
Original Article, 127 - 135Tag this article
The so-called non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) account a very heterogeneous group of tumors. The rarity of each histotype prevents the performance of clinical trials on a single tumor type, and consequently, they have to be analyzed as a group. As definitive evidences about treatment are lacking, the aim of the study is to evaluate the prognosis of NRSTS after a multidisciplinary approach, and to test clinical outcomes of surgical reconstructions. It is performed a retrospective analysis of 33 consecutive pediatric-age patients affected by NRSTS seen at a single institution over a 15-year period. Patients were treated using the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol, where surgery plays the main role and chemo- and radiotherapy are variable associated on the basis of the subtype of tumor, yet leaving wide gaps. Thirty-nine percent of patients received only surgical treatment, 33% patients received chemotherapy associated to surgery, only 3% had radiotherapy and surgery and the residual 24% underwent both chemo- and radiotherapy associated to surgery. The overall survival of our series was 91% at 1 year, 86% at 5 years, 81% at 10 years. The local free recurrence survival was 93% at 1 year, 85% at 5 years and 10 years. The metastasis free survival was 78% at 1 year, 75% at 5 years and 10 years. Limb-salvage surgery was possible in the majority of cases (97%) and functional outcomes were generally good, with only a 15% of patients complaining functional deficit. The results are generally satisfactory and in line with the poor literature available; they underline the primary importance of surgery and a multidisciplinary approach. Moreover, reconstructive procedures and functional outcomes must not be left out because life expectancy in these patients is steadily increasing.
KEY WORDS: Non-Rhadbomyosarcoma Soft Tissue Sarcomas; soft tissues; pediatrics; ortopedic oncology.