Ferrari Andrea
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G Venezian, 1 20133 Milano MI, Italy.
Expert Rev Anticancer Ther. 2008 Jun;8(6):929-38. doi: 10.1586/14737140.8.6.929.
The definition of nonrhabdomyosarcoma soft-tissue sarcomas includes a varied group of malignant soft part tumors that can occur in childhood, but the majority are entities typically observed in adult age. Similar to their adult counterparts, pediatric nonrhabdomyosarcoma soft-tissue sarcomas are usually considered scarcely sensitive to chemotherapy, but treatment strategies for these tumors have changed to some degree in recent years, and multiple-modality treatments that also include chemotherapy have increasingly been attempted. Subsets of patients with specific histological subtypes and prognostic variables have been thought likely to benefit from chemotherapy. The recent development of new molecular treatment approaches to specific tumor targets may enable the current limits of systemic therapies to be overcome in the near future, possibly identifying specific agents tailored to each histotype. While awaiting these developments, however, a better use of standard chemotherapy may prove important in improving the cure rate for these patients.
非横纹肌肉瘤软组织肉瘤的定义包括一组多样的恶性软组织肿瘤，这些肿瘤可发生于儿童期，但大多数是典型见于成人的实体瘤。与成人的非横纹肌肉瘤软组织肉瘤相似，儿童非横纹肌肉瘤软组织肉瘤通常被认为对化疗几乎不敏感，但近年来这些肿瘤的治疗策略已在一定程度上发生了变化，包括化疗在内的多模式治疗越来越多地被尝试。具有特定组织学亚型和预后变量的患者亚组被认为可能从化疗中获益。针对特定肿瘤靶点的新分子治疗方法的最新进展可能使目前全身治疗的局限性在不久的将来得以克服，有可能确定针对每种组织学类型的特定药物。然而，在等待这些进展的同时，更好地使用标准化疗可能对提高这些患者的治愈率很重要。
