Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS)

SummaryNonrhabdmyosarcoma soft tissue sarcoma (NRSTS) is a heterogenous group of tumors analyzed as a unique group because of the rarity of each histopathological subtype. Initial information available has been obtained from adult series or pediatric case studies. Recent reports by several multi-center groups have been published that evaluated prognostic factors and treatment protocols.Established prognostic factors include tumor grade, invasiveness, size, and intergroup rhabdomyosarcoma study (IRS) group. Identification of sarcoma-specific chromosomal translocations has allowed for more accurate definitive diagnosis. Some may have prognostic significance and may offer as potential therapeutic targets. Surgery remained important, as many of the tumors are scarcely chemosensitive. With the development of multimodalities, surgical management has evolved over the years. Patients with localized unresectable disease are managed with surgery +/− radiotherapy with very good results. Localized unresectable disease is intermediate in behavior and prognosis. Patients are treated with neoadjuvant chemotherapy, +/− radiotherapy, followed by delayed surgical resection. Prognosis in patients with metastatic disease has been poor despite combination chemotherapy with surgical resection. The addition of ifosfamide and doxorubicin in the chemotherapy regimes has provided some improvement.With the lack of new active agents, we will depend on advances in molecular techniques to develop novel targeted therapies, better molecular markers and histology-specific trials to evaluate this group of tumors.