Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort
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 c...
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Wiley
2025-05-01
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| Online Access: | https://doi.org/10.1002/cam4.70973 |
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| author | Joseph Sia Stephanie Tan Narayan Mohanakrishnan Kelvin Yu Samuel Y. Ngan Sarat Chander Jayesh Desai Jeremy Lewin Anne Hamilton Stephen J. Luen David E. Gyorki Hayden Snow Claudia DiBella Sarah O'Reilly‐Harbidge Lisa M. Orme Julie Chu Susie Bae |
| author_facet | Joseph Sia Stephanie Tan Narayan Mohanakrishnan Kelvin Yu Samuel Y. Ngan Sarat Chander Jayesh Desai Jeremy Lewin Anne Hamilton Stephen J. Luen David E. Gyorki Hayden Snow Claudia DiBella Sarah O'Reilly‐Harbidge Lisa M. Orme Julie Chu Susie Bae |
| author_sort | Joseph Sia |
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| description | 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. |
| format | Article |
| id | doaj-art-d0f06bd6c1f14313a2f2f3e22be99781 |
| institution | OA Journals |
| issn | 2045-7634 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Wiley |
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| series | Cancer Medicine |
| spelling | doaj-art-d0f06bd6c1f14313a2f2f3e22be997812025-08-20T02:38:09ZengWileyCancer Medicine2045-76342025-05-011410n/an/a10.1002/cam4.70973Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian CohortJoseph Sia0Stephanie Tan1Narayan Mohanakrishnan2Kelvin Yu3Samuel Y. Ngan4Sarat Chander5Jayesh Desai6Jeremy Lewin7Anne Hamilton8Stephen J. Luen9David E. Gyorki10Hayden Snow11Claudia DiBella12Sarah O'Reilly‐Harbidge13Lisa M. Orme14Julie Chu15Susie Bae16Department of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaSir Peter MacCallum Department of Oncology University of Melbourne Melbourne AustraliaDepartment of Medical Oncology Peter MacCallum Cancer Centre Melbourne AustraliaSir Peter MacCallum Department of Oncology University of Melbourne Melbourne AustraliaSir Peter MacCallum Department of Oncology University of Melbourne Melbourne AustraliaSir Peter MacCallum Department of Oncology University of Melbourne Melbourne AustraliaSir Peter MacCallum Department of Oncology University of Melbourne Melbourne AustraliaDivision of Cancer Surgery Peter MacCallum Cancer Centre Melbourne AustraliaDivision of Cancer Surgery Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Medical Oncology Peter MacCallum Cancer Centre Melbourne AustraliaDepartment of Radiation Oncology Peter MacCallum Cancer Centre Melbourne AustraliaSir Peter MacCallum Department of Oncology University of Melbourne Melbourne AustraliaABSTRACT 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.https://doi.org/10.1002/cam4.70973active surveillanceaggressive fibromatosisclinical studydesmoid tumorspatterns of practiceradiotherapy |
| spellingShingle | Joseph Sia Stephanie Tan Narayan Mohanakrishnan Kelvin Yu Samuel Y. Ngan Sarat Chander Jayesh Desai Jeremy Lewin Anne Hamilton Stephen J. Luen David E. Gyorki Hayden Snow Claudia DiBella Sarah O'Reilly‐Harbidge Lisa M. Orme Julie Chu Susie Bae Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort Cancer Medicine active surveillance aggressive fibromatosis clinical study desmoid tumors patterns of practice radiotherapy |
| title | Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort |
| title_full | Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort |
| title_fullStr | Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort |
| title_full_unstemmed | Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort |
| title_short | Trends in Practice Patterns and Clinical Outcomes for Desmoid Tumors: A Large Single‐Institutional Australian Cohort |
| title_sort | trends in practice patterns and clinical outcomes for desmoid tumors a large single institutional australian cohort |
| topic | active surveillance aggressive fibromatosis clinical study desmoid tumors patterns of practice radiotherapy |
| url | https://doi.org/10.1002/cam4.70973 |
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