Synovial sarcoma in children: Surgical lessons from a single institution and review of the literature

BACKGROUND: Synovial sarcomas are malignant high-grade, soft-tissue neoplas ms that account for 7% to 8% of all malignant soft-tissue tumors and are th e most common nonrhabdomyosarcoma soft-tissue sarcomas in pediatric patient s. STUDY DESIGN: A retrospective review of the records of children younger tha n 17 years with synovial, sarcoma treated at the University of Texas MD And erson Cancer Center from 1966 until 1999 was undertaken. Primary site, tumo r size, tumor margins, surgical treatment, adjuvant therapy, local and dist ant recurrence, and survival were recorded for 42 patients. Overall surviva l (OS) and progression-free survival (PFS) rates were calculated by the Kap lan-Meier method. The PFS and OS comparisons were performed using the log-r ank test. RESULTS: Forty-four patients were identified, but two patients were exclude d because of incomplete records. The median followup duration for the 42 pa tients was 8.8 years (range 0.2 to 22.4 years). The 5-year progression-free survival and overall survival rates were 75.6% and 87.7%, respectively. El even patients were dead and four others had progressed but were alive witho ut evidence of disease after further therapy. Intergroup Rhabdomyosarcoma S tudy (IRS) grouping and tumor invasiveness were found to be significant pro gnostic indicators (p < 0.01 and p = 0.02, respectively). Patients with ini tial gross total resection (IRS Groups I and II) and noninvasive tumors (T1 ) were most likely to have prolonged PFS and OS. Patients with small tumors (< 5 cm) (p = 0.09) had better PFS and OS. Adjuvant radiation therapy appe ared to be of benefit, and chemotherapy did not seem to impact PFS or OS. T umors greater than or equal to 5 cm are associated with increased risk of l ocal recurrence and distant metastases. CONCLUSIONS: Complete resection with clear, yet not necessarily large, marg ins remains the treatment of choice for synovial sarcoma in children. Adjuv ant radiation therapy should possibly be considered in patients with dear m argins (IRS Group I) and in patients with microscopic residual tumor (IRS G roup II). Chemotherapy did not seem to impact PFS or OS. Lymph nodes should be evaluated for local regional disease. (J Am Coll Surg 2001;192:305-313. (C) 2001 by the American College of Surgeons).