The Desmoid Tumor Research Foundation

December 2018

Long-term outcomes for patients with desmoid fibromatosis treated with radiation therapy: a 10-year update and re-evaluation of the role of radiation therapy for younger patients

Author Information

Bishop AJ(1), Zarzour MA(2), Ratan R(2), Torres KE(3), Feig BW(3), Wang WL(4), Lazar AJ(4), Moon BS(5), Roland CL(3), Guadagnolo BA(6).

(1)Dept of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX. (2)Dept of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX (3)Dept of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX. (4)Dept of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX. (5)Dept of Orthopedic Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX. (6)Dept of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX; Health Services Research, The University of Texas MDAnderson Cancer Center, Houston, TX.

Publication

Int J Radiat Oncol Biol Phys

Summary

We reviewed the records of 209 consecutive patients with desmoid fibromatosis treated with RT, either alone or as combined modality therapy (CMT) with surgery, at our institution from 1965 to 2015. Median follow-up time was 98 months (range, 1-509 months). The 5-year and 10-year LC was 71% and 69%, respectively. 59 patients (28%) experienced a local recurrence at a median time of 23 months (interquartile range, 15-38 months). Among all patients, on multivariable analysis, adjusting for anatomic site, size, age, treatment era (>2005 vs. ≤ 2005), treatment approach (RT alone vs. CMT), and an interaction between age and treatment, we found only age ≤30 yrs (HR 2.94, P=0.005, 95% CI 1.38-6.27) and large tumor size >10cm (HR 2.51, P=0.03, 95% CI 1.09-5.78) to be correlated with poorer LC. Notably, for patients receiving RT alone the 5-year LC was 43% for patients ≤30 yrs vs 75% for >30 yrs (P<0.001). On multivariable analyses, for patients receiving RT alone, the only factor associated with inferior LC was age ≤30 yrs (HR 2.87, P=0.001, 95% CI 1.51-5.47). The same was true for patients treated with CMT; age ≤30 yrs was the only factor associated with inferior LC (HR 5.36, P=0.01, 95% CI 1.40-20.58). Among all patients with desmoid fibromatosis, RT is an effective local therapy for tumor control. However, young patients ≤ 30 yrs have notably high rates of local recurrence regardless of treatment strategy, which requires further study. Treatment decisions should be risk-adapted by large referral centers with multidisciplinary expertise in desmoid management.

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