Desmoid fibromatosis: MRI features of response to systemic therapy
- 589 Downloads
Imaging criteria for measuring the response of desmoid fibromatosis to systemic therapy are not well established. We evaluated a series of patients with desmoids who underwent systemic therapy to document magnetic resonance imaging (MRI) features associated with a positive clinical response.
Materials and methods
This Institutional Review Board-approved retrospective study included 23 patients (mean age 40.5) with 29 extra-abdominal tumors. Therapeutic regimens included cytotoxic chemotherapy (n = 19), targeted therapy (n = 3), and nonsteroid anti-inflammatory drugs (NSAIDS; n = 1). Clinical effects were categorized as progressive disease, stable, or partial response. Maximum tumor dimension (Dmax), approximate tumor volume (VTumor), and quantitative tumor T2 hyperintensity and contrast enhancement (relative to muscle) for pre- and post-treatment MRIs were compared.
Three lesions progressed, 5 lesions were stable, whereas 21 showed a clinical response. Dmax decreased more in responders (mean −11.0 %) than in stable/progressive lesions (mean −3.6 and 0 % respectively, p = 0.28, ANOVA); by Response Evaluation Criteria in Solid Tumors (RECIST 1.1) 27 out of 29 lesions were “stable,” including the 3 progressive lesions. In responders, VTumor change averaged −29.4 %, but −19.2 % and +32.5 % in stable and progressive lesions respectively (p = 0.002, ANOVA); by 3D criteria 14 out of 29 lesions showed a partial response. T2 hyperintensity decreased by 50–54 % in partial response/stable disease, but only by 10 % in progressive lesions (p = 0.049, t test). Changes in contrast enhancement ranged from −23 % to 0 %, but were not statistically significant among response groups (p = 0.37). Change in T2 hyperintensity showed a positive correlation with volumetric change (r = 0.40).
Decreases in volume and T2 hyperintensity reflect the positive response of desmoid fibromatosis to systemic therapy; RECIST 1.1 criteria are not sensitive to clinically determined tumor response.
KeywordsDesmoid fibromatosis MRI Response criteria 3D Extra-abdominal Systemic therapy
Pooja J. Sheth: data collection, manuscript writing, and editing; Spencer del Moral: data collection; Breelyn A. Wilky: consulting oncologist, assisted with study design; Jonathan C. Trent: consulting oncologist, editing, provided patients for review, assisted with study design; Jonathan Cohen: consulting oncologist, editing, provided patients for review; Andrew E. Rosenberg: consulting pathologist, editing, assisted with study design; H. Thomas Temple: consulting surgeon, editing, materials/methods development; Ty K. Subhawong: primary investigator, manuscript writing and editing, developed study design.
Compliance with ethical standards
Conflicts of interest
The authors declare that they have no conflicts of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.
- 1.Fletcher CDM, Bridge JA, Hogendoorn P, Martens F. WHO classification of tumours of soft tissue and bone, 4th ed. 2013; Geneva: WHO.Google Scholar
- 14.Choi H, Charnsangavej C, Faria SC, et al. Correlation of computed tomography and positron emission tomography in patients with metastatic gastrointestinal stromal tumor treated at a single institution with imatinib mesylate: proposal of new computed tomography response criteria. J Clin Oncol. 2007;25(13):1753–9.CrossRefPubMedGoogle Scholar
- 18.Evans J. Straightforward statistics for the behavioral sciences. Pacific Grove: Brooks/Cole Publishing; 1996.Google Scholar
- 19.Kasper B, Baumgarten C, Bonvalot S, et al. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise - a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer. 2015;51(2):127–36.CrossRefPubMedGoogle Scholar