Prognostic Factors Influencing Progression-Free Survival Determined From a Series of Sporadic Desmoid Tumors: A Wait-and-See Policy According to Tumor Presentation

Authors:
  • ADArmelle Dufresne
    Armelle Dufresne
  • BBBinh Bui
    Binh Bui
  • PTPhilippe Terrier
    Philippe Terrier
  • DRDominique Ranchere-Vince
    Dominique Ranchere-Vince
  • SBSylvie Bonvalot
    Sylvie Bonvalot
  • ESEberhard Stoeckle
    Eberhard Stoeckle
  • LGLouis Guillou
    Louis Guillou
  • ACAxel Le Cesne
    Axel Le Cesne
  • OOOdile Oberlin
    Odile Oberlin
  • VBVéronique Brouste
    Véronique Brouste

Abstract
Purpose Desmoid tumors are mesenchymal fibroblastic/myofibroblastic proliferations with locoregional aggressiveness and high ability to recur after initial treatment. We present the results of the largest series of sporadic desmoid tumors ever published to determine the prognostic factors of these rare tumors. Patients and Methods Four hundred twenty-six patients with a desmoid tumor at diagnosis were included, and the following parameters were studied: age, sex, delay between first symptoms and diagnosis, tumor size, tumor site, previous history of surgery or trauma in the area of the primary tumor, surgical margins, and context of abdominal wall desmoids in women of child-bearing age during or shortly after pregnancy. We performed univariate and multivariate analysis for progression-free survival (PFS). Results In univariate analysis, age, tumor size, tumor site, and surgical margins (R2 v R0/R1) had a significant impact on PFS. PFS curves were not significantly different for microscopic assessment of surgical resection quality (R0 v R1). In multivariate analysis, age, tumor size, and tumor site had independent values. Three prognostic groups for PFS were defined on the basis of the number of independent unfavorable prognostic factors (0 or 1, 2, and 3). Conclusion This study clearly demonstrates that there are different prognostic subgroups of desmoid tumors that could benefit from different therapeutic strategies, including a wait-and-see policy.

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