Clinical features and outcomes of young patients with head and neck non-rhabdomyosarcoma soft tissue sarcomas

Background. The history, prognostic factors, and outcome of young patients with head and neck non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) have not been adequately characterized. Methods. Medical records of 58 patients with head and neck NRSTS treated at St. Jude Children's Research Hospital were reviewed. Results. The majority of tumors were ≤5 cm and high grade. Lymph node and/or distant metastases were present in 17% at presentation. Patients received a combination of surgery, chemotherapy, and radiotherapy. The 10-year event-free and survival rates were 53.1%67.3% and 63.2%67.1%, respectively. Features associated with inferior survival included high histologic grade (p5.006), tumor diameter ≥5 cm (p <.001), invasiveness (p <.001), and incomplete resection at diagnosis (p5.005). Conclusion. Most head and neck NRSTS in young patients are small, high grade, and nonmetastatic. The outcome is poor compared to NRSTS at other anatomic sites. Innovative approaches to local control and improved systemic therapy are needed.