Advances in the Management of Pediatric Sarcomas
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SARCOMAS (SR PATEL, SECTION EDITOR)
Advances in the Management of Pediatric Sarcomas Fiorela N. Hernandez Tejada 1 & Alejandro Zamudio 1 & Mario L. Marques-Piubelli 2 & Branko Cuglievan 1 & Douglas Harrison 1 Accepted: 5 November 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review The prognosis of pediatric patients who present with metastatic or recurrent sarcomas remains poor. In this review, we summarize the advances in the management of metastatic and relapsed pediatric sarcoma by highlighting recent and future clinical trials. Recent Findings Research into the identification of novel therapies for refractory pediatric sarcomas continues to advance. Outcomes have not improved in several decades underlying a need for improved understanding of the biology behind these tumors and the identification of novel therapeutic molecular targets that can be exploited pharmacologically. Multiple challenges remain for novel therapy in sarcomas such as the selection of effective targets, management of toxicities, and the tumor microenvironment. Summary Many unique challenges remain in the treatment of patients with refractory pediatric sarcomas. Multiple strategies and targets are under investigation that hold promise. Keywords Sarcomas . Pediatric sarcomas . Immunotherapy . Therapy
Introduction Sarcomas are tumors of mesenchymal origin believed to arise from bone or soft tissue precursors. While broadly rare— accounting for only about 1% of all cancers in the general population—they represent approximately 13% of cancers in patients who are under the age of 20 [1]. Pediatric sarcomas are largely divided between those that arise from bone, and those that arise from soft tissue. The most common malignant tumors of bone are osteosarcoma and Ewing sarcoma (ES), while rhabdomyosarcoma (RMS) is the most common sarcoma of soft tissue. Other non-RMS soft tissue sarcomas such as desmoplastic small round cell and synovial sarcoma, become more common as children age into adolescence and young adulthood [2]. Current treatment regimens rely on a
combination of systemic chemotherapy, surgery, and radiation therapy and have resulted in 5-year event-free survival (EFS) rates of 60–70% depending on the specific histologic diagnosis and presence of metastatic disease. Patients with metastatic disease have a poor prognosis with 5-year EFS rates in the 20–30% range. Patients with disease recurrence fare even poorer with EFS less than 20% [3, 4]. In this review, we summarize recent advances in the management of pediatric sarcomas with a brief overview of current therapeutic options followed by a review of active and recently closed clinical trials that have explored novel biologic targets and immunotherapy.
Osteosarcoma This article is part of the Topical Collection on Sarcomas * Douglas Harrison [email protected] 1
Division of Pediatrics, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA
2
Department of Translational Molecular Pathology,
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