Evidence-based MR imaging follow-up strategy for desmoid-type fibromatosis

To propose a follow-up strategy for desmoid-type fibromatosis (DF) based on tumor growth behavior and the signal on T2-weighted MRI.

We retrospectively reviewed 296 MRI studies of 34 patients with histologically proven DF. In each study, tumor volume and T2 signal relatively normal striated muscle were assessed. Volume variation and monthly growth rates were analyzed to determine lesion growth behavior (progressing versus stable/regressing lesions). Growth behavior was correlated with T2 signal, tumor location, β-catenin status, treatment strategy, and follow-up duration. Interobserver variability of volume measurements and interobserver measurement variation ratio were assessed.

There were 25 women and 9 men with a mean age of 39.9 ± 19 (4-73) years. Mean follow-up time in the patients included was 55 ± 41 (12-148) months. In progressing lesions, the mean average monthly growth ratio was 10.9 ± 9.2 (1.1-42.5) %. Interobserver variability of volume measurements was excellent (ICC = 0.96). Mean interobserver measurement variation ratio was 20.4 ± 23.6%. The only factor correlated with tumor growth behavior was T2 signal ratio (p < 0.0001). Seventeen out of 34 (50%) patients presented a signal change over the threshold of 1 during follow-up. There were five occurrences of secondary growth after a period of stability with a mean delay until growth of 38.2 ± 44.2 (17-116) months.

DF growth rate was quantitatively assessed. A threshold for volume variation detection was established. DF growth behavior was significantly related to T2 signal. An evidence-based follow-up strategy is proposed.

• In progressing desmoid fibromatosis, the mean average monthly growth ratio was 10.9 ± 9.2%. • Lesions with muscle/tumor T2 signal ratios lower than 1 tended to be stable or regress over time. • Given the interobserver measurement variability and MRI in-plane spatial resolution, a variation higher than 42.6% in tumor volume is required to confirm punctual progression.