Current management of pediatric soft tissue sarcomas

Abstract

Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma (RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.

Call for improved design and reporting in soft tissue sarcoma studies: A systematic review and meta‐analysis of chemotherapy and survival outcomes in resectable STS

“…In children, who account for up to 15% of all STS diagnoses, embryonal RMS – extremely rare in adults – predominates, with RMS accounting for over 50% of pediatric STS diagnoses . The remaining diagnoses are non‐RMS tumors, most commonly fibrosarcomas and extra‐osseous Ewing's tumors, which are both relatively rare in adults . Furthermore, even within the pediatric population, there are nuanced changes in outcomes for RMS with increasing age .…”

“…29,30 The remaining diagnoses are non-RMS tumors, most commonly fibrosarcomas and extra-osseous Ewingʼs tumors, which are both relatively rare in adults. 29,31 Furthermore, even within the pediatric population, there are nuanced changes in outcomes for RMS with increasing age. 30 These known differences between pediatric and adult STS populations suggest that these groups may not be meaningfully studied together.…”

Elastofibroma Dorsi: Review of Literature with Radiological Approach

“…From these, 60% are rhabdomiosarcomas, being the most prevalent tumor. It may grow in any part of the body, nevertheless the most common areas are in the head, neck, genitourinary system and extremities [28] (Figure 7).…”

Primary Site and Other Prognostic Variables Effects on Survival in Pediatric Synovial Sarcoma

“…Primary soft tissue sarcomas most commonly present in joints, nerves, muscles, fat, deep skin tissues, and blood vessels [1]. Overall the prognosis is better in pediatric soft tissue sarcomas compared to adults [2]. Synovial sarcomas (SS) are an aggressive type of soft tissue sarcoma that represents approximately 10% of soft tissue sarcomas [2].…”

“…Overall the prognosis is better in pediatric soft tissue sarcomas compared to adults [2]. Synovial sarcomas (SS) are an aggressive type of soft tissue sarcoma that represents approximately 10% of soft tissue sarcomas [2]. SS primarily present in the lower extremities, but have been found in many other sites including the abdomen, thorax, and head and neck [3][4][5][6][7][8][9].…”