{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T17:04:24Z","timestamp":1786122264977,"version":"build-2736575974"},"reference-count":31,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background: Desmoid tumors (DTs) are rare, locally aggressive fibroblastic neoplasms with highly heterogeneous clinical behavior. The present work constitutes the second part of a two-part project, following our previously published multidisciplinary review of the diagnostic and therapeutic landscape of DTs. It provides a comprehensive analysis of our institutional experience as a national reference center for sarcoma. We aim to describe real-world diagnostic pathways, management strategies, and clinical outcomes in a high-volume cohort. Methods: We conducted a retrospective cohort study that included patients diagnosed with DT at our center between 2014 and 2024. Demographic, clinical, molecular, treatment, and outcome data were collected. Management strategies were analyzed according to tumor location, symptoms, progression patterns, and multidisciplinary decision-making. Outcomes included response rates, event-free survival (EFS), need for active treatment, response to systemic therapy, and recurrence after local treatments. Results: A total of 109 patients were included (median age 36.8 years; 56.9% women). Somatic CTNNB1 mutations were identified in 23 of 29 tested patients, predominantly T41A, while germline alterations were found in 18 patients, mainly in APC. Initial management was conservative in 40.4% of patients and active in 59.6%, primarily through surgery. After a median follow-up of 41.5 months, 44.9% of patients experienced disease progression. Among patients managed with active surveillance, spontaneous regression occurred in 22.2%, and 58% remained treatment-free. Surgical relapse occurred in 35.8% of patients undergoing upfront resection, with major postoperative complications limited to externally operated cases. Cryoablation achieved radiological responses in most evaluable patients, while systemic therapies showed clinical activity but relevant toxicity, particularly with tyrosine kinase inhibitors. The median EFS for the whole cohort was 57 months. Conservative initial management and R1\/2 surgical margins were independently associated with worse EFS. Conclusions: Our results support a personalized, multidisciplinary management strategy for DTs, prioritizing conservative approaches when appropriate and reserving active treatments for progressive or symptomatic disease. Outcomes achieved in a specialized referral center are comparable to those reported in large international retrospective series, underscoring the value of expert multidisciplinary care in optimizing DT management.<\/jats:p>","DOI":"10.3390\/cancers18020305","type":"journal-article","created":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T14:58:54Z","timestamp":1768834734000},"page":"305","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Experience of a Referral Center with Desmoid Tumors, Part 2: A Retrospective Analysis of 109 Cases"],"prefix":"10.3390","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0935-8298","authenticated-orcid":false,"given":"Rosa","family":"Alvarez Alvarez","sequence":"first","affiliation":[{"name":"Medical Oncology Department, Instituto de Investigacion Sanitaria Gregorio Mara\u00f1on, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"Agra Pujol","sequence":"additional","affiliation":[{"name":"Pathology Department, Hospital General Universitario Gregorio Mara\u00f1on, Complutense University, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-5878-5309","authenticated-orcid":false,"given":"Marta","family":"Arregui Valles","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Instituto de Investigacion Sanitaria Gregorio Mara\u00f1on, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8851-3948","authenticated-orcid":false,"given":"Francisco","family":"Alijo","sequence":"additional","affiliation":[{"name":"Pathology Department, Hospital General Universitario Gregorio Mara\u00f1on, Complutense University, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-8008-0917","authenticated-orcid":false,"given":"Adriana","family":"Fern\u00e1ndez Gonzalo","sequence":"additional","affiliation":[{"name":"Radiology Department, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Guti\u00e9rrez","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Instituto de Investigacion Sanitaria Gregorio Mara\u00f1on, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5413-8449","authenticated-orcid":false,"given":"Pablo","family":"Lozano Lominchar","sequence":"additional","affiliation":[{"name":"General Surgery and Surgical Oncology Department, Hospital General Universitario Gregorio Mara\u00f1on, Complutense University, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9922-0078","authenticated-orcid":false,"given":"Cristina","family":"Mata Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Gregorio Mara\u00f1\u00f3n Materno Infantil Hospital, 28009 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydia","family":"Mediavilla Santos","sequence":"additional","affiliation":[{"name":"Orthopedic Surgery and Traumatology Department, Musculoskeletal Oncology Section, Hospital General Universitario Gregorio Mara\u00f1on, Complutense University, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2172-8960","authenticated-orcid":false,"given":"Ulrike","family":"Novo","sequence":"additional","affiliation":[{"name":"Radiology Department, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-0175-6509","authenticated-orcid":false,"given":"Marina","family":"Santos","sequence":"additional","affiliation":[{"name":"Radiation Oncology Department, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-7200-8982","authenticated-orcid":false,"given":"Guillermo","family":"Hern\u00e1ndez Torrado","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Instituto de Investigacion Sanitaria Gregorio Mara\u00f1on, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henar","family":"Carpintero Garc\u00eda","sequence":"additional","affiliation":[{"name":"Orthopedic Surgery and Traumatology Department, Hospital General Universitario Gregorio Mara\u00f1on, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Guti\u00e9rrez-Ortiz","family":"de la Tabla","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Hospital Universitario Infanta Leonor, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,1,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1634\/theoncologist.2010-0281","article-title":"Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease","volume":"16","author":"Kasper","year":"2011","journal-title":"Oncologist"},{"key":"ref_2","unstructured":"Fletcher, C., Bridge, J.A., Hogendoorn, P.C.W., and Mertens, F. 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