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Skeletal Radiology

, Volume 45, Issue 10, pp 1365–1373 | Cite as

Desmoid fibromatosis: MRI features of response to systemic therapy

  • Pooja J. Sheth
  • Spencer del Moral
  • Breelyn A. Wilky
  • Jonathan C. Trent
  • Jonathan Cohen
  • Andrew E. Rosenberg
  • H. Thomas Temple
  • Ty K. Subhawong
Scientific Article

Abstract

Objective

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.

Results

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).

Conclusion

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.

Keywords

Desmoid fibromatosis MRI Response criteria 3D Extra-abdominal Systemic therapy 

Notes

Authors’ roles

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.

Ethical approval

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.

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Copyright information

© ISS 2016

Authors and Affiliations

  • Pooja J. Sheth
    • 1
  • Spencer del Moral
    • 2
  • Breelyn A. Wilky
    • 2
  • Jonathan C. Trent
    • 2
  • Jonathan Cohen
    • 3
  • Andrew E. Rosenberg
    • 4
  • H. Thomas Temple
    • 5
  • Ty K. Subhawong
    • 1
  1. 1.Department of RadiologyUniversity of Miami Miller School of Medicine/Jackson Memorial HospitalMiamiUSA
  2. 2.Division of Hematology/Oncology, Department of MedicineUniversity of Miami Miller School of Medicine/Sylvester Comprehensive Cancer CenterMiamiUSA
  3. 3.Oncology and Radiation AssociatesMiamiUSA
  4. 4.Department of PathologyUniversity of Miami Miller School of MedicineMiamiUSA
  5. 5.Center for Orthopedic InnovationsMiamiUSA

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