Pharmacotherapy for pediatric soft-tissue sarcomas

INTRODUCTION: Soft-tissue sarcomas are rare, but they represent about 8% of all malignancies in pediatric age. Developing a multidisciplinary approach to treatment based on risk stratification has led to a dramatic improvement in survival, but a plateau has been reached with current treatment options in the last 20 years. Chemotherapy is usually effective for rhabdomyosarcoma and should be seen as the keystone of its treatment, while non-rhabdomyosarcoma soft-tissue sarcomas are still generally considered scarcely chemosensitive. AREAS COVERED: An overview of current, emerging and possible future medical therapies for pediatric soft-tissue sarcomas is provided. Insight into different chemotherapeutic strategies based on risk stratification for rhabdomyosarcoma and non-rhabdomyosarcoma soft tissue sarcomas in pediatric age is given. EXPERT OPINION: Integrating systemic therapy with local treatments (surgery and/or radiotherapy) is complex and requires adequate experience, which can only be assured by referral institutions. Future challenges include identifying novel targeted therapies and optimizing treatment protocols for customized patient care.