Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G Venezian 1, Milan, Italy.
Expert Opin Pharmacother. 2011 Mar;12(4):517-31. doi: 10.1517/14656566.2011.524926.
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.
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.
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.
软组织肉瘤较为罕见，但在儿童时期的所有恶性肿瘤中占比约为 8%。通过基于风险分层的多学科治疗方法，生存率得到了显著提高，但在过去 20 年中，当前的治疗方案已经达到了一个瓶颈。化疗通常对横纹肌肉瘤有效，应将其视为治疗的基石，而非横纹肌肉瘤软组织肉瘤仍然被普遍认为对化疗的敏感性较低。
本文提供了儿童软组织肉瘤当前、新兴和未来可能的医学治疗方法的概述。深入了解了基于风险分层的横纹肌肉瘤和儿童时期非横纹肌肉瘤软组织肉瘤的不同化疗策略。
将系统治疗与局部治疗（手术和/或放疗）相结合非常复杂，需要有足够的经验，而这只能由转诊机构来保证。未来的挑战包括确定新的靶向治疗方法和优化治疗方案，以实现个性化的患者护理。
