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Background: ARMS is considered to be one of the most aggressive pediatric malignancies. Adults ith ARMS notoriously experience inferior outcomes. Our goal as to define our adult population ith ARMS and revie their outcomes on front-line systemic therapies. Methods: Adult ARMS pts over the age of 18 years y treated at MDACC from 2004 to 2015 ere identified from our registry. Descriptive statistics and survival analysis ere performed via multivariate analysis. Results: 43 pts ere identified. Median age at diagnosis as 31 y range R 18 to 69 y . 51 percent ere male. 26 pts 60 percent ere Caucasian, 10 pts 23 percent ere Latino, 5 pts 12 percent ere Asian and 2 pts 5 percent ere African-American. The most common primary site as the parameningeal space 25 pts; 58 percent . The majority ere classified ith IRSG stage IV 37 percent or III disease 47 percent . The majority ere classified ith group IV 37 percent or III 56 percent . By COG clinical risk, 27 pts 63 percent and 16 pts 37 percent ere intermediate and high, respectively. N1 disease as noted in 31 pts 72 percent . M1 disease as observed in 16 pts 37 percent . 36 pts 84 percent had an unfavorable primary site. 60 percent of pts had primary tumors 5 cm. FOXO1 fusions ere present in 30 pts 70 percent , negative in 4 pts 9 percent , and not evaluated in 9 21 percent . Most common chemotherapy regimens employed doxorubicin 26 pts; 49 percent , actinomycin-D 12 pts; 28 percent , both 3 pts; 7 percent , or other 2 pts; 5 percent . A median of 8 cycles R: 1 20 cycles ere received. RECIST Responses included CR 21 percent , PR 26 percent , and SD 7 percent . 30 pts 70 percent received radiation therapy for local control. Median OS as 32 months R: 2 to 97 months . By multivariate analysis, m status P = 0.007 and clinical risk P = 0.007 ere associated ith statistically significant differences in survival. An inferior OS as noted in pts receiving doxorubicin-based chemotherapy P = 0.037 , though an associated increase in absolute tumor size as noted in this group P = 0.057 . Only 21 percent of pts ere disease free at last follo up or death.Conclusions: Adults ith ARMS represent a poorly defined group ith an unmet need for improved treatment. Clinical trials are needed to improve the outcomes for this rare population of patients.,J Clin Oncol 34, 2016 suppl; abstr 11069 00:00.0,Sarcoma
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