Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single-Institutional Australian Cohort

Sia, J; Tan, S; Mohanakrishnan, N; Yu, K; Ngan, SY; Chander, S; Desai, J; Lewin, J; Hamilton, A; Luen, SJ; Gyorki, DE; Snow, H; DiBella, C; O&#39;Reilly-Harbidge, S; Orme, LM; Chu, J; Bae, S

Author(s): Sia, J; Tan, S; Mohanakrishnan, N; Yu, K; Ngan, SY; Chander, S; Desai, J; Lewin, J; Hamilton, A; Luen, SJ; Gyorki, DE; Snow, H; DiBella, C; O'Reilly-Harbidge, S; Orme, LM; Chu, J; Bae, S;
Details: Publication Year 2025-05,Volume 14,Issue #10,Page e70973
Journal Title: Cancer Medicine
Publication Type: Research article
Abstract: BACKGROUND: Desmoid tumors (DT) are rare, locally aggressive neoplasms that affect a young population and have a tendency for recurrence. There is sparse contemporary real-world data to guide practice for DT. Here, we report on a large cohort of DT patients, describing patterns of care and clinical outcomes. METHODS: Data on DT patients first seen between 2010 and 2021 were extracted from a prospective database and supplemented with a retrospective review of hospital records. Trends in treatment use were analyzed using the Cochran-Armitage test. Time-to-next intervention (TTNI) was estimated with the Kaplan-Meier method. Imaging response was categorized using the RECIST v1.1 criteria. RESULTS: A total of 135 patients, 265 treatment episodes were analyzed. Median follow-up was 4.3 years. The common tumor sites were abdominal wall (27%), upper limb (20%), lower limb (16%), and intra-abdominal (15%). Over time, the proportion of patients receiving no upfront treatment was stable (2010-2013: 31%, 2014-2017: 35%, 2018-2021: 29%; p = 0.5), but there was increasing first-line use of NSAID/tamoxifen (7%, 41%, 47%; p < 0.001), and decreasing first-line use of radiotherapy (35%, 14%, 4%; p < 0.001) and surgery (28%, 8%, 18%; p < 0.05). At 5 years, the proportion not requiring treatment switch was highest following surgery (72%), radiotherapy (66%), and no upfront therapy (52%). 12% and 5% of patients without treatment achieved partial and complete imaging responses at 2 years. CONCLUSION: We highlight the heterogeneity and trends in DT management over a 12-year period, affirming the role of active surveillance, radiotherapy, and surgery in selected patients. Medical therapies are evolving and may significantly influence the DT management paradigm.
Publisher: Wiley
Keywords: Humans; *Desmoid Tumors/therapy/pathology; Female; Male; Adult; Middle Aged; Young Adult; Adolescent; Retrospective Studies; *Practice Patterns, Physicians'/trends; Australia/epidemiology; Child; Treatment Outcome; Aged; Child, Preschool; active surveillance; aggressive fibromatosis; clinical study; desmoid tumors; patterns of practice; radiotherapy; surgery; targeted therapy
Department(s): Radiation Oncology; Medical Oncology; Surgical Oncology
Publisher's Version: https://doi.org/10.1002/cam4.70973
Open Access at Publisher's Site: https://doi.org/10.1002/cam4.70973
Terms of Use/Rights Notice: Refer to copyright notice on published article.

Creation Date: 2025-07-08 04:31:26

Last Modified: 2025-07-08 04:32:02