ArticleClinical cancer research : an official journal of the American Association for Cancer Research2022
Systemic Chemotherapies Retain Antitumor Activity in Desmoid Tumors Independent of Specific Mutations in CTNNB1 or APC: A Multi-institutional Retrospective Study.
Article in Clinical cancer research : an official journal of the American Association for Cancer Research, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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17 citing papers in PubMed, 13 citations in OpenAlex.
- Phase III Trial of Pegylated Liposomal Doxorubicin for Patients with Advanced and Refractory Desmoid Tumors.Clinical cancer research : an official journal of the American Association for Cancer Research · 2026Trial
- Real-World Characterization and Treatment Patterns of Patients with Desmoid Tumors at an Academic Center in the United States.Cancer research communications · 2026Article
- Genotype-guided conservative management of mesenteric desmoid tumors: A case report of intermediate-region APC mutations.Medicine · 2026Article
- Surgical Management of Desmoid Tumors-Patient Selection, Timing, and Approach.Current oncology (Toronto, Ont.) · 2025Review
- Updates and controversies for desmoids in familial adenomatous polyposis.Familial cancer · 2025Review
- Article
- Case Report: Nirogacestat therapy induces rapid response in a patient with refractory, life-threatening desmoid tumor.Frontiers in oncology · 2025Article
- Efficacy and safety of systemic treatment for progressive and refractory desmoid tumor: a systematic review and Bayesian network meta-analysis.Discover oncology · 2024Article
- Favorable clinical efficacy of cytotoxic chemotherapy in patients with progressive desmoid tumors: a retrospective real-world study.International journal of clinical oncology · 2024Article
- Case report: Rapidly progressive desmoid tumor after surgery for esophagogastric junction cancer and slowly progressive primary desmoid tumor: a report of two cases and literature review.Frontiers in oncology · 2024Article
- Efficacy and safety of anlotinib in patients with desmoid fibromatosis: a retrospective analysis.Frontiers in oncology · 2024Article
- Clinical results of active surveillance for extra-abdominal desmoid-type fibromatosis.Cancer medicine · 2023Article
- Current therapies and future prospective for locally aggressive mesenchymal tumors.Frontiers in oncology · 2023Review
- MRI T2 mapping assessment of T2 relaxation time in desmoid tumors as a quantitative imaging biomarker of tumor response: preliminary results.Frontiers in oncology · 2023Article
- Bilateral breast desmoid-type fibromatosis, case report and literature review.Gland surgery · 2022Article
- Article
- Multimodality Imaging Assessment of Desmoid Tumors: The Great Mime in the Era of Multidisciplinary Teams.Journal of personalized medicine · 2022Review
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27 authors at 13 institutions in 2 countries.
Funding
Abstract
purposeDetermine whether specific CTNNB1 or APC mutations in patients with desmoid tumor were associated with differences in clinical responses to systemic treatments. EXPERIMENTAL
designWe established a multi-institutional dataset of previously treated patients with desmoid tumor across four U.S. sarcoma centers, including demographic and clinicopathologic characteristics, treatment regimens, and clinical and radiographic responses. CTNNB1 or APC mutation status was determined from prior pathology records, or archival tissue was requested and analyzed by Sanger sequencing and/or next-generation sequencing. Evaluable patients with mutation results were analyzed to determine clinical progression-free survival (cPFS), RECIST 1.1 PFS (rPFS), time to next treatment (TTNT), and overall survival (OS). Kaplan-Meier analysis and Cox proportional hazards regression were performed to identify differences in cPFS, rPFS, TTNT, and OS by mutation subtype, desmoid tumor location, and treatment regimen.
resultsA total of 259 evaluable patients were analyzed for at least one of the survival outcomes, with 177 patients having mutation data. First- and second-line cPFS, rPFS, and TTNT were not significantly affected by mutation subtype; however, APC-mutant desmoid tumors demonstrated nonstatistically significant inferior outcomes. Extremity/trunk desmoid tumor location and treatment with doxorubicin-based, methotrexate/vinca alkaloids and sorafenib regimens were associated with better clinical outcomes compared with surgery or "other" therapies, including estrogen-receptor blockade and imatinib. OS was significantly worse with APC or CTNNB1 negative/other mutations.
conclusionsMutation subtype did not affect responses to specific systemic therapies. APC mutations and nonextremity desmoid tumor locations remain prognostic for worse outcomes, and earlier initiation of systemic therapy for these higher-risk desmoid tumors should be prospectively evaluated. See related commentary by Greene and Van Tine, p. 3911.
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