Role of chemotherapy in pediatric nonrhabdomyosarcoma soft-tissue sarcomas

Abstract

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.