Upfront observation versus radiation for adult XXXX .

@entity1 @entity816 @entity1313
BACKGROUND: Although @entity816 accounts for up to 40% of all childhood @entity1313 , it is a rare disease in adults. Consequently, there are few mature data on the impact of up-front treatment options after surgery that include observation or adjuvant radiotherapy. METHODS: Ten @entity1 and 20 @entity1 were identified who were diagnosed with @entity816 from 1971 to 2007 and were retrospectively reviewed. The median @entity1 age was 30 years (range, 18-64 years), and the median follow-up was 87 months (range, 16-420 months). Initial surgery included biopsy (10% of @entity1 ), subtotal resection (57% of @entity1 ), or gross-total resection (33% of @entity1 ). Nineteen @entity1 were observed postoperatively, whereas 11 @entity1 received up-front postoperative adjuvant radiotherapy (50 grays in 25 fractions). No @entity1 received adjuvant or concurrent chemotherapy. Progression-free survival (PFS) and overall survival (OS) were calculated using the Kaplan-Meier method. Differences between survival curves were analyzed with the log-rank test. RESULTS: For the entire cohort, the 5-year and 10-year OS rates were 95% and 85%, respectively, and the 5-year and 10-year PFS rates were 63% and 35%, respectively. The median PFS was 8.4 years. Initial radiation, compared with observation, did not have an impact on OS but significantly improved PFS. The 5-year PFS rate for @entity1 who were observed versus those who received radiation was 42% versus 91%, respectively; and, at 10 years, the PFS rate was 17% versus 60%, respectively (P = .005). @entity1 who progressed after observation (11 of 19 @entity1 ) received various salvage therapies, resulting in a 2-year PFS rate of 68% compared with 33% for @entity1 who progressed after initial radiation (3 of 11 @entity1 ) and were salvaged with either chemotherapy or surgery (P = .1). CONCLUSIONS: Adjuvant radiotherapy for @entity816 significantly prolonged PFS at both 5 years and 10 years compared with observation. However, equivalent OS was observed, which reflected the efficacy of salvage therapies.
