Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTS) represent a subgroup of sarcomas that encompass more than 50 distinct histologies. All are rare, but some are more common in patients younger than 20 years of age. The management of patients with many histologies overlap. However, this review will focus on issues unique to a select few NRSTS that are most common in pediatric and adolescent patients. Here, we will discuss the recent advances in the diagnosis, surgical management, and treatment of NRSTS. Adequate surgical local control of the primary tumor is a critical component of the treatment strategy will be emphasized in this review because it determines local and distant recurrence.
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