Nonrhabdomyosarcoma soft tissue sarcomas in children

Nonrhabdomyosarcoma soft tissue sarcomas are very rare tumors in the pediatric population and consist of a heterogeneous collection of subtypes. They can occur anywhere in the body, with the extremities the most common anatomic site. In the initial evaluation of a soft tissue mass, proper radiographic evaluation is best performed with magnetic resonance (MR) imaging, while tissue for diagnosis should be obtained with a well planned incisional biopsy. Complete surgical resection remains the cornerstone of therapy, but it is now recognized that multi-modal strategies incorporating surgery, radiotherapy, and chemotherapy should be studied for incompletely resected tumors and those with poor prognostic indicators.