OncoTargets and Therapy (Jun 2020)
Management of Refractory Pediatric Sarcoma: Current Challenges and Future Prospects
Abstract
Deepam Pushpam,1,* Vikas Garg,1,* Sandip Ganguly,2 Bivas Biswas2 1Department of Medical Oncology, AIIMS, New Delhi, India; 2Department of Medical Oncology, Tata Medical Center, Kolkata, India*These authors contributed equally to this workCorrespondence: Bivas BiswasDepartment of Medical Oncology, Tata Medical Center, 14 MAR [EW] New Town, Rajarhat, Kolkata 700160, IndiaTel +91033 6605 7628Email [email protected]: Paediatric sarcomas are a heterogeneous group of disorders constituting bone sarcoma and various soft tissue sarcomas. Almost one-third of these presents with metastasis at baseline and another one-third recur after initial curative treatment. There is a huge unmet need in this cohort in terms of curative options and/or prolongation of survival. In this review, we have discussed the current treatment options, challenges and future strategies of managing relapsed/refractory paediatric sarcomas. Upfront risk-adapted treatment with multidisciplinary management remains the main strategy to prevent future recurrence or relapse of the disease. In the case of limited local and/or systemic relapse or late relapse, initial multimodality management can be administered. In treatment-refractory cases or where cure is not feasible, the treatment options are limited to novel therapeutics, immunotherapeutic approach, targeted therapies, and metronomic therapies. A better understanding of disease biology, mechanism of treatment refractoriness, identifications of driver mutation, the discovery of novel targeted therapies, cellular vaccine and adapted therapies should be explored in relapsed/refractory cases. Close national and international collaboration for translation research is needed to fulfil the unmet need.Keywords: paediatric sarcoma, immunotherapy, targeted therapy, relapsed sarcoma, osteosarcoma, disease biology
