{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T22:42:40Z","timestamp":1789684960601,"version":"4.0.1"},"reference-count":27,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T00:00:00Z","timestamp":1748563200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Current Oncology"],"abstract":"<jats:p>Background: Desmoid fibromatosis (DF) is a rare, locally aggressive soft tissue tumour with unpredictable clinical behaviour. Historically, treatment has involved surgery; however, contemporary guidelines, such as those from the Desmoid Tumour Working Group, advocate active surveillance. This article reviews current perspectives on DF, focusing on epidemiology, pathogenesis, treatment strategies, emerging research directions and cost effectiveness based on our experience at the West of Scotland Musculoskeletal Oncology Service, Glasgow Royal Infirmary (GRI). Methodology: We reviewed 101 patients diagnosed with desmoid fibromatosis between 2010 and 2024. A review of patient records was conducted to gather information on demographics, date of diagnosis, prior treatment, treatment initiation, intervention types, imaging intervals, follow-up duration, recurrence rate for surgery and other intervention, and discharge timelines. All data was systematically organized and analyzed to assess our outcomes. Results: Out of 101 patients with DF in the study, 66% were females. The most common site of primary tumour was lower extremity (39.6%) followed by near equal distribution in upper extremity and trunk. Out of the total cases, 72 (71.2%) were successfully managed with active surveillance involving serial imaging and clinical reviews in accordance with European guidelines. A total of 22 patients (21%) received treatment: 10 underwent surgery alone, 2 had surgery combined with radiotherapy, 8 received only radiotherapy, 1 was treated with hormonal therapy and 1 participated in a trial with Nirogacestat. Of the seven remaining patients, six had unplanned surgery outside followed by active surveillance at GRI. One patient was on alternative treatment modality, homeopathy. The average number of MRI scans per patient was 3.11, with many patients requiring significantly more imaging. MRI surveillance varies significantly in desmoid tumours due to their heterogeneous behaviour. Active or symptomatic tumours often require more frequent scans (every 3\u20136 months), while stable cases may need only imaging annually or just clinical monitoring. Recurrence was noted in eight patients, all of which were related to prior surgery. The total combined cost of imaging and appointments exceeds \u00a36500 per patient in active surveillance. Conclusions: We conclude that most patients with desmoid fibromatosis in our cohort were effectively treated with active surveillance, consistent with current European guidelines. Surgical management of desmoid fibromatosis in our cohort is historic and has shown a significant recurrence risk. Our study proposes a revised follow-up protocol that significantly reduces costs without compromising on patient care. We suggest a two-year surveillance period for stable disease with patient-initiated return to reduce unnecessary clinic visits, imaging and healthcare costs.<\/jats:p>","DOI":"10.3390\/curroncol32060320","type":"journal-article","created":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T06:14:27Z","timestamp":1748585667000},"page":"320","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Evaluating Management of Extra-Abdominal Desmoid Fibromatosis: A Retrospective Analysis of Treatments, Outcomes and Recurrence Patterns"],"prefix":"10.3390","volume":"32","author":[{"given":"Vidhi","family":"Saraf","sequence":"first","affiliation":[{"name":"Wolfson Medical School, Glasgow University, Glasgow G12 8QQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2854-5435","authenticated-orcid":false,"given":"Hariharan","family":"Triplicane Dwarakanathan","sequence":"additional","affiliation":[{"name":"Glasgow Royal Infirmary, Glasgow G4 0SF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-2882-3746","authenticated-orcid":false,"given":"Al-Muaayad","family":"Al-Abri","sequence":"additional","affiliation":[{"name":"Wolfson Medical School, Glasgow University, Glasgow G12 8QQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ioanna","family":"Nixon","sequence":"additional","affiliation":[{"name":"The Beatson West of Scotland Cancer Centre, Glasgow G12 0YN, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Vaughan","sequence":"additional","affiliation":[{"name":"Glasgow Royal Infirmary, Glasgow G4 0SF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashish","family":"Mahendra","sequence":"additional","affiliation":[{"name":"Glasgow Royal Infirmary, Glasgow G4 0SF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1165-6585","authenticated-orcid":false,"given":"Sanjay","family":"Gupta","sequence":"additional","affiliation":[{"name":"Glasgow Royal Infirmary, Glasgow G4 0SF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,5,30]]},"reference":[{"key":"ref_1","unstructured":"Fletcher, C.D.M., Bridge, J.A., Hogendoorn, P., and Mertens, F. (2013). WHO Classification of Tumours of Soft Tissue and Bone (IARC WHO Classification of Tumours), International Agency for research on Cancer. [4th ed.]."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"x158","DOI":"10.1093\/annonc\/mds298","article-title":"The Treatment of Desmoid Tumors: A Stepwise Clinical Approach","volume":"23","author":"Bonvalot","year":"2012","journal-title":"Ann. Oncol. Off. J. Eur. Soc. Med. Oncol."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Litchman, C. (2012). Clinical Presentation of Desmoid Tumors. Desmoid Tumors, Springer.","DOI":"10.1007\/978-94-007-1685-8"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1038","DOI":"10.1016\/j.crad.2015.04.015","article-title":"Desmoid-Type Fibromatosis","volume":"70","author":"Otero","year":"2015","journal-title":"Clin. Radiol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e422","DOI":"10.1016\/j.crad.2014.06.006","article-title":"Soft-Tissue Masses in the Abdominal Wall","volume":"69","author":"Bashir","year":"2014","journal-title":"Clin. Radiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"215810","DOI":"10.1155\/2012\/215810","article-title":"Imaging Features of Superficial and Deep Fibromatoses in the Adult Population","volume":"2012","author":"Walker","year":"2012","journal-title":"Sarcoma"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1016\/j.ejrad.2008.10.012","article-title":"Can the MRI Signal of Aggressive Fibromatosis Be Used to Predict Its Behavior?","volume":"69","author":"Castellazzi","year":"2009","journal-title":"Eur. J. Radiol."},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Simonetti, I., Bruno, F., Fusco, R., Cutolo, C., Setola, S.V., Patrone, R., Masciocchi, C., Palumbo, P., Arrigoni, F., and Picone, C. (2022). Multimodality Imaging Assessment of Desmoid Tumors: The Great Mime in the Era of Multidisciplinary Teams. J. Pers. Med., 12.","DOI":"10.3390\/jpm12071153"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"W1008","DOI":"10.2214\/AJR.11.6657","article-title":"A to Z of Desmoid Tumors","volume":"197","author":"Shinagare","year":"2011","journal-title":"Am. J. Roentgenol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.acra.2022.08.009","article-title":"Primary Soft-Tissue Lymphomas: MRI Features Help Discriminate From Other Soft-Tissue Tumors","volume":"30","author":"Spinnato","year":"2023","journal-title":"Acad. Radiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2399","DOI":"10.1093\/annonc\/mdx323","article-title":"An Update on the Management of Sporadic Desmoid-Type Fibromatosis: A European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)\/Soft Tissue and Bone Sarcoma Group (STBSG)","volume":"28","author":"Kasper","year":"2017","journal-title":"Ann. Oncol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/j.ejca.2019.11.013","article-title":"The Management of Desmoid Tumours: A Joint Global Consensus-Based Guideline Approach for Adult and Paediatric Patients","volume":"127","author":"Alman","year":"2020","journal-title":"Eur. J. Cancer"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.ocl.2005.08.004","article-title":"Desmoid Tumors and Current Status of Management","volume":"37","author":"Hosalkar","year":"2006","journal-title":"Orthop. Clin. N. Am."},{"key":"ref_14","first-page":"186","article-title":"Sporadic Extra Abdominal Wall Desmoid-Type Fibromatosis: Surgical Resection Can Be Safely Limited to a Minority of Patients","volume":"51","author":"Colombo","year":"2015","journal-title":"Eur. J. Cancer Oxf. Engl. 1990"},{"key":"ref_15","first-page":"18","article-title":"Active Surveillance in Desmoid-Type Fibromatosis: A Systematic Literature Review","volume":"137","author":"Timbergen","year":"2020","journal-title":"Eur. J. Cancer Oxf. Engl. 1990"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3397","DOI":"10.2217\/fon-2021-0003","article-title":"Desmoid Fibromatosis: Is the Current Picture Changing?","volume":"17","author":"Pandrowala","year":"2021","journal-title":"Future Oncol. Lond. Engl."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"3027","DOI":"10.1002\/cncr.34332","article-title":"Evolving Strategies for Management of Desmoid Tumor","volume":"128","author":"Riedel","year":"2022","journal-title":"Cancer"},{"key":"ref_18","unstructured":"(2024, December 20). Four Different Treatment Strategies in Aggressive Fibromatosis: A Systematic Review, Available online: https:\/\/pubmed.ncbi.nlm.nih.gov\/30069502\/."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1001\/jamaoncol.2024.1805","article-title":"Current Management of Desmoid Tumors: A Review","volume":"10","author":"Kasper","year":"2024","journal-title":"JAMA Oncol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"100562","DOI":"10.1016\/j.ctarc.2022.100562","article-title":"Current Management and Recent Progress in Desmoid Tumors","volume":"31","author":"Zhou","year":"2022","journal-title":"Cancer Treat. Res. Commun."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1016\/j.suc.2022.05.005","article-title":"The Evolving Management of Desmoid Fibromatosis","volume":"102","author":"Prendergast","year":"2022","journal-title":"Surg. Clin. N. Am."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"4036","DOI":"10.1245\/s10434-012-2634-6","article-title":"Management and Recurrence Patterns of Desmoids Tumors: A Multi-Institutional Analysis of 211 Patients","volume":"19","author":"Peng","year":"2012","journal-title":"Ann. Surg. Oncol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"5213","DOI":"10.1002\/cncr.33233","article-title":"Desmoid Tumors: To Treat or Not to Treat, That Is the Question","volume":"126","author":"Kasper","year":"2020","journal-title":"Cancer"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"184","DOI":"10.3390\/cancers4010184","article-title":"Desmoid Tumors in Pregnant and Postpartum Women","volume":"4","author":"Robinson","year":"2012","journal-title":"Cancers"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1097\/CCO.0000000000000374","article-title":"Adult Desmoid Tumors: Biology, Management and Ongoing Trials","volume":"29","author":"Penel","year":"2017","journal-title":"Curr. Opin. Oncol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e005282","DOI":"10.1136\/bmjopen-2014-005282","article-title":"Effect of a Redesigned Fracture Management Pathway and \u2018Virtual\u2019 Fracture Clinic on ED Performance","volume":"4","author":"Vardy","year":"2014","journal-title":"BMJ Open"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1177\/00369330211008598","article-title":"A Virtual Multi-Disciplinary Meeting Is a Cost-Effective Method of Triaging Referrals to a Regional Musculoskeletal Oncology Service","volume":"66","author":"Alanie","year":"2021","journal-title":"Scott. Med. J."}],"container-title":["Current Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1718-7729\/32\/6\/320\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T17:43:52Z","timestamp":1760031832000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1718-7729\/32\/6\/320"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,30]]},"references-count":27,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2025,6]]}},"alternative-id":["curroncol32060320"],"URL":"https:\/\/doi.org\/10.3390\/curroncol32060320","relation":{},"ISSN":["1718-7729"],"issn-type":[{"value":"1718-7729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,5,30]]}}}