Treatment Concepts and Challenges in Nonrhabdomyosarcoma Soft Tissue Sarcomas

Abstract

Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) encompass a heterogeneous group of mesenchymal tumors with more than 50 histologic variants. The incidence of NRSTS is greater than rhabdomyosarcoma; however, each histologic type is rare. The treatment schema for all NRSTSs is largely surgical. The treatment is a risk-adapted approach based on tumor size, localization, tumor grade, and presence of metastases. Low-grade tumors are mainly managed by surgery alone, whereas for high-grade tumors a multimodal treatment concept is necessary. The multimodal treatment consists of tumor biopsy, chemotherapy, local treatment (surgery ± radiotherapy), and immunotherapy in selected conditions.

Keywords: Congenital infantile fibrosarcoma; Desmoid tumor; Desmoplastic small round cell tumor; Myofibroblastic tumor; Pediatric nonrhabdomyosarcoma; Peripheral nerve sheath tumor; Synovial sarcoma.