title: Desmoid fibromatosis: MRI features of response to systemic therapy.
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.
  
  
  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).
  
  
  CONCLUSIONS
  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.
authors: Department of Radiology, University of Miami Miller School of Medicine/Jackson Memorial Hospital, 1611 NW 12th Avenue, JMH WW 279, Miami, FL, 33136, USA.
authors: Pooja J
authors: PJ
authors: Sheth
authors: Division of Hematology/Oncology, Department of Medicine, University of Miami Miller School of Medicine/Sylvester Comprehensive Cancer Center, Miami, FL, 33136, USA.
authors: Spencer
authors: S
authors: Del Moral
authors: Division of Hematology/Oncology, Department of Medicine, University of Miami Miller School of Medicine/Sylvester Comprehensive Cancer Center, Miami, FL, 33136, USA.
authors: Breelyn A
authors: BA
authors: Wilky
authors: Division of Hematology/Oncology, Department of Medicine, University of Miami Miller School of Medicine/Sylvester Comprehensive Cancer Center, Miami, FL, 33136, USA.
authors: Jonathan C
authors: JC
authors: Trent
authors: Oncology and Radiation Associates, 1321 NW 14th Street, Suite 207, Miami, FL, 33125, USA.
authors: Jonathan
authors: J
authors: Cohen
authors: Department of Pathology, University of Miami Miller School of Medicine, Miami, FL, 33136, USA.
authors: Andrew E
authors: AE
authors: Rosenberg
authors: Center for Orthopedic Innovations, 3659 S. Miami Avenue, Suite 4008, Miami, FL, 33133, USA.
authors: H Thomas
authors: HT
authors: Temple
authors: Department of Radiology, University of Miami Miller School of Medicine/Jackson Memorial Hospital, 1611 NW 12th Avenue, JMH WW 279, Miami, FL, 33136, USA. tsubhawong@med.miami.edu.
authors: Ty K
authors: TK
authors: Subhawong
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage*; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D016903:Drug Monitoring / Q000379:methods*; D005260:Female; D018222:Desmoid Tumors / Q000000981:diagnostic imaging* / Q000188:drug therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D000970:Antineoplastic Agents
keywords: 3D; Desmoid fibromatosis; Extra-abdominal; MRI; Response criteria; Systemic therapy