{ "abstract": "Background\n\nActive surveillance is recommended as the first-line management for desmoid-type fibromatosis (DF); however, data on DF of the head and neck (H&N) and shoulder regions are limited.Methods\n\nWe examined patients with DF located in the H&N and shoulder regions, comparing baseline characteristics and event-free survival (EFS). A subgroup analysis was performed among patients initially managed with active surveillance.Results\n\nH&N accounted for 4% of all DF cases, and shoulder accounted for 5%. Pain (62% vs. 53% and 33%, respectively; p = 0.01) and functional impairment (29% vs. 0% and 6%, respectively; p = 0.002) were significantly more frequent in shoulder DF compared with H&N DF and DF at other sites. Regardless of initial management, the 3-year EFS rates for H&N and shoulder were similar to those for other locations (p = 0.74). Among patients initially managed with active surveillance, the 3-year EFS rates for H&N and shoulder were similar to that for DF at other locations (p = 0.63).Conclusion\n\nPain and functional impairment differed significantly by location; however, the outcomes of both H&N and shoulder DF did not differ significantly from those of DF at other locations.Trial registration\n\nThis investigation was registered under the clinical trial registration number NCT02867033.", "abstract_shown": true, "affiliations": [ "Department of Medical Oncology, Centre Oscar Lambret, Lille, France.", "CHU Lille, ULR 2694-Metrics: Evaluation des Technologies de Santé et des Pratiques Médicales, Univ. Lille, Lille, France.", "Department of Surgical Oncology, Institut Curie, Paris, France.", "Université Paris Sciences et Lettres, Paris, France.", "Clinical Research Department, Centre Oscar Lambret, Lille, France.", "Department of Medical Oncology, Institut Bergonié, Bordeaux, France.", "Department of Medical Oncology, Gustave Roussy, Villejuif, France.", "Department of Medical Oncology, Strasbourg University Hospital, Strasbourg, France.", "Department of Medical Oncology, Centre Jean Perrin, Clermont-Ferrand, France.", "Medical Oncology Department, Centre Henri Becquerel, Rouen, France.", "Medical Oncology Department, Public Assistance-Hospitals of Marseille, La Timone Hospital, Marseille, France.", "Medical Oncology Department, Institut Paoli-Calmettes, Aix-Marseille Univ., Marseille, France.", "Medical Oncology Department, Centre René Gauduchau, Saint Herblain, France.", "Department of Medical Oncology, Institut de Cancérologie de Montpellier, Montpellier, France.", "INSERM U1194, IRCM, Univ. Montpellier, Montpellier, France.", "Department of Medical Oncology, AP-HP, Cochin Hospital, Paris, France.", "Department of Medical Oncology, Institut Curie, Paris, France.", "INSERM U830, Institut Curie Research Center, Paris, France.", "Department of Medical Oncology, Institut Claudius Regaud and Institut Universitaire de Cancerologie, Oncopole, Toulouse, France.", "Department of Medical Oncology, Centre Léon Bérard, Université Claude Bernard, Lyon, France." ], "article_type": "research article", "arxiv": null, "author_total": 20, "authors": [ { "affs": [ 0, 1 ], "name": "Nicolas Penel", "orcid": "0000-0001-5243-1548" }, { "affs": [ 2, 3 ], "name": "Sylvie Bonvalot", "orcid": "0000-0001-9726-9732" }, { "affs": [ 4 ], "name": "Julien Elices-Diez", "orcid": "0009-0001-3983-5713" }, { "affs": [ 5 ], "name": "Antoine Italiano" }, { "affs": [ 6 ], "name": "Benjamin Verret" }, { "affs": [ 7 ], "name": "Jean-Emmanuel Kurtz" }, { "affs": [ 8 ], "name": "Pascale Dubray-Longeras" }, { "affs": [ 9 ], "name": "Cécile Guillemet" }, { "affs": [ 10 ], "name": "Florence Duffaud", "orcid": "0000-0002-2057-3557" }, { "affs": [ 11 ], "name": "François Bertucci", "orcid": "0000-0002-0157-0959" }, { "affs": [ 4 ], "name": "Julien Thery", "orcid": "0000-0001-9783-5925" }, { "affs": [ 4 ], "name": "Marie Vanseymortier", "orcid": "0009-0008-0481-7049" }, { "affs": [ 12 ], "name": "Emmanuelle Bompas" }, { "affs": [ 13, 14 ], "name": "Nelly Firmin", "orcid": "0000-0001-5839-6261" }, { "affs": [ 15 ], "name": "Sixtine De Percin", "orcid": "0000-0001-5773-0205" }, { "affs": [ 4 ], "name": "Jennifer Wallet" }, { "affs": [ 16, 17 ], "name": "Sophie Piperno-Neumann" }, { "affs": [ 18 ], "name": "Thibaud Valentin" }, { "affs": [ 19 ], "name": "Jean-Yves Blay" }, { "affs": [ 4 ], "name": "Marie-Cécile Le Deley", "orcid": "0000-0002-2946-4592" } ], "bibtex": "https://research.draconitic.com/paper/45vkngn5nbbkd.bib", "cited_by": 0, "doi": "10.1002/cam4.72302", "fulltext_url": "https://europepmc.org/articles/PMC13686588", "id": "45vkngn5nbbkd", "issns": [ "2045-7634" ], "landing_url": "https://europepmc.org/article/MED/42905937", "language": "eng", "licence": "CC-BY-NC-ND", "licence_label": "CC BY-NC-ND", "licence_source": "article", "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/", "metadata_licence": null, "native_subjects": [ [ "lcc", "RC254-282" ], [ "openalex", "2745|Soft tissue tumor case studies" ] ], "openalex": "W7223143525", "pdf_url": "https://europepmc.org/articles/PMC13686588?pdf=render", "pmcid": "PMC13686588", "pmid": "42905937", "published": "2026-10-01", "publisher": "Wiley", "ris": "https://research.draconitic.com/paper/45vkngn5nbbkd.ris", "sources": [ { "first_seen": "2026-10-30T12:19:57Z", "native_id": "MED:42905937", "source": "europepmc", "url": "https://europepmc.org/article/MED/42905937" }, { "first_seen": "2026-10-30T13:44:31Z", "native_id": "65afb7f3ef674410829372a27752bdbb", "source": "doaj", "url": "https://doi.org/10.1002/cam4.72302" } ], "subjects": [ { "field": "medicine", "origin": "lcc:RC254-282", "subfield": "oncology" } ], "title": "Head & Neck and Shoulder Desmoid-Type Fibromatosis: Presentation and Natural History of These Ultra-Rare Locations.", "title_text": "Head & Neck and Shoulder Desmoid-Type Fibromatosis: Presentation and Natural History of These Ultra-Rare Locations.", "type": "peer-reviewed", "updated_at": "2026-10-30T13:44:31Z", "url": "https://research.draconitic.com/paper/45vkngn5nbbkd", "venue": "Cancer medicine", "versions": [] }