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Penel, Nicolas; Bonvalot, Sylvie; Bimbai, André-Michel; Meurgey, Alexandra; Le Loarer, François; Salas, Sébastien; Piperno-Neumann, Sophie; Chevreau, Christine; Boudou-Rouquette, Pascaline; Dubray-Longeras, Pascale; Kurtz, Jean-Emmanuel; Guillemet, Cécile; Bompas, Emmanuelle; Italiano, Antoine; Le Cesne, Axel; Orbach, Daniel; Thery, Julien; Le Deley, Marie-Cécile; Blay, Jean-Yves; Mir, Olivier

Lack of Prognostic Value of CTNNB1 Mutation Profile in Desmoid-Type Fibromatosis

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  • Media type: E-Article
  • Title: Lack of Prognostic Value of CTNNB1 Mutation Profile in Desmoid-Type Fibromatosis
  • Contributor: Penel, Nicolas; Bonvalot, Sylvie; Bimbai, André-Michel; Meurgey, Alexandra; Le Loarer, François; Salas, Sébastien; Piperno-Neumann, Sophie; Chevreau, Christine; Boudou-Rouquette, Pascaline; Dubray-Longeras, Pascale; Kurtz, Jean-Emmanuel; Guillemet, Cécile; Bompas, Emmanuelle; Italiano, Antoine; Le Cesne, Axel; Orbach, Daniel; Thery, Julien; Le Deley, Marie-Cécile; Blay, Jean-Yves; Mir, Olivier
  • imprint: American Association for Cancer Research (AACR), 2022
  • Published in: Clinical Cancer Research
  • Language: English
  • DOI: 10.1158/1078-0432.ccr-21-4235
  • ISSN: 1078-0432; 1557-3265
  • Keywords: Cancer Research ; Oncology
  • Origination:
  • Footnote:
  • Description: <jats:title>Abstract</jats:title> <jats:sec> <jats:title>Purpose:</jats:title> <jats:p>This prospective nationwide cohort study aimed to investigate desmoid-type fibromatosis (DF) outcomes, focusing on the prognostic value of CTNNB1 mutations.</jats:p> </jats:sec> <jats:sec> <jats:title>Experimental Design:</jats:title> <jats:p>ALTITUDES (NCT02867033) was a nationwide prospective cohort study of DF diagnosed between January 2016 and December 2020. At diagnosis, CTNNB1 molecular alterations were identified using next-generation sequencing or Sanger sequencing. The primary endpoint was event-free survival (EFS; progression, relapse, or death). We enrolled 628 patients managed by active surveillance, surgical resection, or systemic treatment as first-line therapy.</jats:p> </jats:sec> <jats:sec> <jats:title>Results:</jats:title> <jats:p>Overall, 516 (82.2%) patients [368 females (71.3%), median age 40.3 years (range, 1–89)] were eligible for analysis. In 435 (84.3%) cases, there was one CTNNB1 molecular alteration: p.T41A, p.S45F, or p.S45P. The first-line management was active surveillance in 352 (68.2%), surgical resection in 120 (23.3%), and systemic treatments in 44 (8.5%) patients. CTNNB1 mutation distribution was similar across the three therapeutic groups. The median follow-up period was 24.7 (range, 0.4–59.7) months. The estimated 3-year EFS rate was 66.2% [95% confidence interval (CI), 60.5%–71.2%]. DF harboring p.S45F was significantly associated with male sex (P = 0.03), non-abdominal wall sites (P = 0.05), pain (P = 0.007), and large tumor size (P = 0.025). CTNNB1 p.S45F mutation was not significantly associated with EFS, either in univariate (HR, 1.06; 95% CI, 0.65–1.73; P = 0.81) or in multivariate analysis (HR, 0.91; 95% CI, 0.55–1.49; P = 0.71).</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions:</jats:title> <jats:p>We found that CTNNB1 mutation profile was associated with unfavorable prognostic factors but was not a prognostic factor for EFS.</jats:p> <jats:p>See related commentary by Greene and Van Tine, p. 3911</jats:p> </jats:sec>
  • Access State: Open Access