{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T14:39:50Z","timestamp":1786372790364,"version":"build-2736575974"},"reference-count":67,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T00:00:00Z","timestamp":1750377600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T00:00:00Z","timestamp":1750377600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Familial Cancer"],"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p>Desmoids are rare non-cancerous fibrous growths with variable behavior ranging from slow indolent growth or even regression, to locally aggressive and progressive tumors that can cause significant morbidity or mortality. Approximately 10\u201315% of patients with familial adenomatous polyposis (FAP) develop desmoid disease, most commonly located in the abdomen, on the abdominal wall, or in limbs. The majority of desmoids in FAP occur after abdominal surgery. Management is quite challenging and employing a multidisciplinary team at a specialized center is important for success. New treatment modalities have emerged, including tyrosine kinase inhibitors, \u03b3-secretase inhibitors, and ablation techniques, complementing the existing repertoire of therapies such as NSAIDs, anti-hormonal therapy, chemotherapy, radiotherapy, and surgical interventions. Surgery remains the treatment of choice for easily resectable abdominal wall desmoids and intra-abdominal desmoids that cause intractable symptoms, or progressive disease despite alternate therapies, or complications from the invasion of nearby organs. When considering prophylactic colectomies in FAP patients, it\u2019s essential to account for the desmoidogenic potential of surgical interventions, especially in high-risk individuals with a positive family history of desmoids, presence of extracolonic manifestations and carriers of certain genotypes. Given the rarity of the disease and the variability in both anatomical presentation and clinical course, desmoids should be managed by a multidisciplinary team capable of coordinating patient specific care and optimizing treatment options.<\/jats:p>","DOI":"10.1007\/s10689-025-00481-9","type":"journal-article","created":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T04:44:45Z","timestamp":1750394685000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Updates and controversies for desmoids in familial adenomatous polyposis"],"prefix":"10.1007","volume":"24","author":[{"given":"Rami James N.","family":"Aoun","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew F.","family":"Kalady","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,6,20]]},"reference":[{"key":"481_CR1","unstructured":"National Comprehensive Cancer Network (2024) Soft Tissue Sarcoma (Version 4. https:\/\/www.nccn.org\/professionals\/physician_gls\/pdf\/sarcoma.pdf"},{"key":"481_CR2","unstructured":"International Agency for Research on Cancer WHO classification of tumours of soft tissue and bone. World Health Organization. Published online 2013."},{"issue":"2","key":"481_CR3","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1016\/j.ejca.2014.11.005","volume":"51","author":"B Kasper","year":"2015","unstructured":"Kasper B, Baumgarten C, Bonvalot S et al (2015) Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients\u2019 and professionals\u2019 expertise\u2013a sarcoma patients EuroNet and European organisation for research and treatment of cancer\/soft tissue and bone sarcoma group initiative. Eur J Cancer 51(2):127\u2013136","journal-title":"Eur J Cancer"},{"key":"481_CR4","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1016\/j.ejca.2019.11.013","volume":"127","author":"B Alman","year":"2020","unstructured":"Alman B, Attia S, Baumgarten C et al (2020) The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer 127:96\u2013107","journal-title":"Eur J Cancer"},{"key":"481_CR5","unstructured":"Kasper B, Baldini EH, Bonvalot S et al Current management of desmoid tumors: A review. JAMA Oncol. Published online 2024."},{"issue":"10","key":"481_CR6","doi-asserted-by":"publisher","first-page":"2399","DOI":"10.1093\/annonc\/mdx323","volume":"28","author":"B Kasper","year":"2017","unstructured":"Kasper B, Baumgarten C, Garcia J et al (2017) 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). Ann Oncol 28(10):2399\u20132408","journal-title":"Ann Oncol"},{"issue":"12","key":"481_CR7","doi-asserted-by":"publisher","first-page":"7943","DOI":"10.1245\/s10434-024-15938-x","volume":"31","author":"AJ Bartholomew","year":"2024","unstructured":"Bartholomew AJ, Rhodin KE, Noteware L et al (2024) Evolution of initial treatment for desmoid tumors. Ann Surg Oncol 31(12):7943\u20137949","journal-title":"Ann Surg Oncol"},{"issue":"6","key":"481_CR8","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1080\/14737167.2023.2203915","volume":"23","author":"MM Fernandez","year":"2023","unstructured":"Fernandez MM, Bell T, Tumminello B, Khan S, Zhou S, Oton AB (2023) Disease and economic burden of surgery in desmoid tumors: a review. Expert Rev Pharmacoeconomics Outcomes Research 23(6):607\u2013618","journal-title":"Expert Rev Pharmacoeconomics Outcomes Research"},{"key":"481_CR9","doi-asserted-by":"publisher","first-page":"102114","DOI":"10.1016\/j.canep.2022.102114","volume":"77","author":"M Anneberg","year":"2022","unstructured":"Anneberg M, Svane HM, Fryzek J et al (2022) The epidemiology of desmoid tumors in Denmark. Cancer Epidemiol 77:102114","journal-title":"Cancer Epidemiol"},{"issue":"4","key":"481_CR10","doi-asserted-by":"publisher","first-page":"900","DOI":"10.1016\/S0022-5223(19)34669-0","volume":"104","author":"JT Brodsky","year":"1992","unstructured":"Brodsky JT, Gordon MS, Hajdu SI, Burt M (1992) Desmoid tumors of the chest wall: a locally recurrent problem. J Thorac Cardiovasc Surg 104(4):900\u2013903","journal-title":"J Thorac Cardiovasc Surg"},{"key":"481_CR11","doi-asserted-by":"crossref","unstructured":"Constantinidou A, Scurr M, Judson I, Litchman C Clinical presentation of desmoid tumors. Desmoid tumors. Published Online 2012:5\u201316","DOI":"10.1007\/978-94-007-1685-8_2"},{"key":"481_CR12","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1016\/j.ejca.2016.02.008","volume":"58","author":"N Penel","year":"2016","unstructured":"Penel N, Coindre JM, Bonvalot S et al (2016) Management of desmoid tumours: a nationwide survey of labelled reference centre networks in France. Eur J Cancer 58:90\u201396","journal-title":"Eur J Cancer"},{"issue":"1","key":"481_CR13","doi-asserted-by":"publisher","first-page":"256","DOI":"10.1002\/ijc.25664","volume":"129","author":"MH Nieuwenhuis","year":"2011","unstructured":"Nieuwenhuis MH, Casparie M, Mathus-Vliegen LM, Dekkers OM, Hogendoorn PC, Vasen HF (2011) A nation\u2010wide study comparing sporadic and Familial adenomatous polyposis\u2010related desmoid\u2010type fibromatoses. Int J Cancer 129(1):256\u2013261","journal-title":"Int J Cancer"},{"issue":"6","key":"481_CR14","first-page":"1193","volume":"59","author":"DM Eccles","year":"1996","unstructured":"Eccles DM, van der Luijt R, Breukel C et al (1996) Hereditary desmoid disease due to a frameshift mutation at codon 1924 of the APC gene. Am J Hum Genet 59(6):1193","journal-title":"Am J Hum Genet"},{"issue":"9","key":"481_CR15","doi-asserted-by":"publisher","first-page":"e24354","DOI":"10.1371\/journal.pone.0024354","volume":"6","author":"E Robanus-Maandag","year":"2011","unstructured":"Robanus-Maandag E, Bosch C, Amini-Nik S et al (2011) Familial adenomatous polyposis-associated desmoids display significantly more genetic changes than sporadic desmoids. PLoS ONE 6(9):e24354","journal-title":"PLoS ONE"},{"issue":"11","key":"481_CR16","doi-asserted-by":"publisher","first-page":"1222","DOI":"10.1111\/j.1463-1318.2010.02345.x","volume":"13","author":"A Sinha","year":"2011","unstructured":"Sinha A, Tekkis P, Gibbons DC, Phillips R, Clark S (2011) Risk factors predicting desmoid occurrence in patients with Familial adenomatous polyposis: a meta-analysis. Colorectal Dis 13(11):1222\u20131229","journal-title":"Colorectal Dis"},{"issue":"2","key":"481_CR17","doi-asserted-by":"publisher","first-page":"102","DOI":"10.1002\/1097-0215(20010320)95:2<102::AID-IJC1018>3.0.CO;2-8","volume":"95","author":"L Bertario","year":"2001","unstructured":"Bertario L, Russo A, Sala P et al (2001) Genotype and phenotype factors as determinants of desmoid tumors in patients with Familial adenomatous polyposis. Int J Cancer 95(2):102\u2013107","journal-title":"Int J Cancer"},{"issue":"3","key":"481_CR18","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1136\/gut.35.3.377","volume":"35","author":"A Gurbuz","year":"1994","unstructured":"Gurbuz A, Giardiello F, Petersen G et al (1994) Desmoid tumours in Familial adenomatous polyposis. Gut 35(3):377\u2013381","journal-title":"Gut"},{"key":"481_CR19","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s40591-015-0038-2","volume":"3","author":"MV Enzo","year":"2015","unstructured":"Enzo MV, Rastrelli M, Rossi CR, Hladnik U, Segat D (2015) The Wnt\/\u03b2-catenin pathway in human fibrotic-like diseases and its eligibility as a therapeutic target. Mol Cell Ther 3:1\u201313","journal-title":"Mol Cell Ther"},{"issue":"10","key":"481_CR20","doi-asserted-by":"publisher","first-page":"606","DOI":"10.1002\/gcc.22272","volume":"54","author":"AM Crago","year":"2015","unstructured":"Crago AM, Chmielecki J, Rosenberg M et al (2015) Near universal detection of alterations in CTNNB1 and Wnt pathway regulators in desmoid-type fibromatosis by whole\u2010exome sequencing and genomic analysis. Genes Chromosom Cancer 54(10):606\u2013615","journal-title":"Genes Chromosom Cancer"},{"issue":"11","key":"481_CR21","doi-asserted-by":"publisher","first-page":"546","DOI":"10.1016\/j.mpdhp.2008.08.004","volume":"14","author":"S Rossi","year":"2008","unstructured":"Rossi S, Laurino L, Dei Tos AP (2008) Desmoid-type fibromatosis: from morphology to molecular genetics. Diagn Histopathology 14(11):546\u2013551","journal-title":"Diagn Histopathology"},{"issue":"47","key":"481_CR22","doi-asserted-by":"publisher","first-page":"6615","DOI":"10.1038\/sj.onc.1203041","volume":"18","author":"S Tejpar","year":"1999","unstructured":"Tejpar S, Nollet F, Li C et al (1999) Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor). Oncogene 18(47):6615\u20136620","journal-title":"Oncogene"},{"issue":"4","key":"481_CR23","first-page":"1437","volume":"45","author":"AG Knudson","year":"1985","unstructured":"Knudson AG (1985) Hereditary cancer, oncogenes, and antioncogenes. Cancer Res 45(4):1437\u20131443","journal-title":"Cancer Res"},{"issue":"10","key":"481_CR24","doi-asserted-by":"publisher","first-page":"1229","DOI":"10.1097\/DCR.0b013e318227e4e8","volume":"54","author":"MH Nieuwenhuis","year":"2011","unstructured":"Nieuwenhuis MH, Lefevre JH, B\u00fclow S et al (2011) Family history, surgery, and APC mutation are risk factors for desmoid tumors in Familial adenomatous polyposis: an international cohort study. Diseases colon rectum 54(10):1229\u20131234","journal-title":"Diseases colon rectum"},{"issue":"6","key":"481_CR25","doi-asserted-by":"publisher","first-page":"716","DOI":"10.1002\/jso.24699","volume":"116","author":"L Koskenvuo","year":"2017","unstructured":"Koskenvuo L, Ristim\u00e4ki A, Lepist\u00f6 A (2017) Comparison of sporadic and FAP-associated desmoid\u2010type fibromatoses. J Surg Oncol 116(6):716\u2013721","journal-title":"J Surg Oncol"},{"issue":"1","key":"481_CR26","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1002\/cncr.35040","volume":"130","author":"P Hohenberger","year":"2024","unstructured":"Hohenberger P, Menge F, Hohenberger R et al (2024) Trauma and sporadic desmoid tumor development: an approach toward real incidence and aspects of causality. Cancer 130(1):51\u201359","journal-title":"Cancer"},{"issue":"11","key":"481_CR27","doi-asserted-by":"publisher","first-page":"1008","DOI":"10.1002\/hed.20467","volume":"28","author":"C Wang","year":"2006","unstructured":"Wang C, Chang Y, Ko J, Cheng C, Yeh C, Lou P (2006) Desmoid tumor of the head and neck. Head Neck: J Sci Specialties Head Neck 28(11):1008\u20131013","journal-title":"Head Neck: J Sci Specialties Head Neck"},{"issue":"10","key":"481_CR28","doi-asserted-by":"publisher","first-page":"768","DOI":"10.1111\/tbj.14276","volume":"27","author":"PA Costa","year":"2021","unstructured":"Costa PA, Marbin S, Costa BMLA et al (2021) A nonrandom association of breast implants and the formation of desmoid tumors. Breast J 27(10):768\u2013775","journal-title":"Breast J"},{"key":"481_CR29","doi-asserted-by":"crossref","unstructured":"Aelvoet AS, Struik D, Bastiaansen BA et al Colectomy and desmoid tumours in Familial adenomatous polyposis: a systematic review and meta-analysis. Familial Cancer Published Online 2022:1\u201311","DOI":"10.1007\/s10689-022-00288-y"},{"issue":"4","key":"481_CR30","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1245\/s10434-016-5380-3","volume":"23","author":"Y Saito","year":"2016","unstructured":"Saito Y, Hinoi T, Ueno H et al (2016) Risk factors for the development of desmoid tumor after colectomy in patients with Familial adenomatous polyposis: multicenter retrospective cohort study in Japan. Ann Surg Oncol 23(4):559\u2013565","journal-title":"Ann Surg Oncol"},{"issue":"3","key":"481_CR31","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1046\/j.1463-1318.1999.00045.x","volume":"1","author":"O Church, McGannon","year":"1999","unstructured":"Church, McGannon O (1999) The clinical course of intra-abdominal desmoid tumours in patients with Familial adenomatous polyposis. Colorectal Dis 1(3):168\u2013173","journal-title":"Colorectal Dis"},{"key":"481_CR32","doi-asserted-by":"publisher","first-page":"1528","DOI":"10.1007\/s10350-005-0018-8","volume":"48","author":"J Church","year":"2005","unstructured":"Church J, Berk T, Boman BM et al (2005) Staging intra-abdominal desmoid tumors in Familial adenomatous polyposis: a search for a uniform approach to a troubling disease. Diseases Colon Rectum 48:1528\u20131534","journal-title":"Diseases Colon Rectum"},{"issue":"3","key":"481_CR33","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1007\/DCR.0b013e3181c52894","volume":"53","author":"M Joyce","year":"2010","unstructured":"Joyce M, Mignanelli E, Church J (2010) Ureteric obstruction in Familial adenomatous polyposis-associated desmoid disease. Diseases colon rectum 53(3):327\u2013332","journal-title":"Diseases colon rectum"},{"issue":"3","key":"481_CR34","doi-asserted-by":"publisher","first-page":"343","DOI":"10.1097\/DCR.0b013e3182a85d9f","volume":"57","author":"JM Church","year":"2014","unstructured":"Church JM, Xhaja X, Warrier SK et al (2014) Desmoid tumors do not prevent proctectomy following abdominal colectomy and ileorectal anastomosis in patients with Familial adenomatous polyposis. Diseases colon rectum 57(3):343\u2013347","journal-title":"Diseases colon rectum"},{"issue":"10","key":"481_CR35","doi-asserted-by":"publisher","first-page":"1762","DOI":"10.1007\/DCR.0b013e3181b5518a","volume":"52","author":"E Elayi","year":"2009","unstructured":"Elayi E, Manilich E, Church J (2009) Polishing the crystal ball: knowing genotype improves ability to predict desmoid disease in patients with Familial adenomatous polyposis. Diseases colon rectum 52(10):1762\u20131766","journal-title":"Diseases colon rectum"},{"issue":"6","key":"481_CR36","doi-asserted-by":"publisher","first-page":"632","DOI":"10.1016\/S0003-4975(10)65358-1","volume":"15","author":"PJ Bolanowski","year":"1973","unstructured":"Bolanowski PJ, Groff DB (1973) Thoracic wall desmoid tumor in a child. Ann Thorac Surg 15(6):632\u2013635","journal-title":"Ann Thorac Surg"},{"key":"481_CR37","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1007\/s11748-006-0068-4","volume":"55","author":"T Nakagiri","year":"2007","unstructured":"Nakagiri T, Koseki M, Nakamoto K, Taniyama K (2007) Paraesophageal mediastinal desmoid tumor: case report. Gen Thorac Cardiovasc Surg 55:125\u2013129","journal-title":"Gen Thorac Cardiovasc Surg"},{"issue":"8","key":"481_CR38","doi-asserted-by":"publisher","first-page":"e1","DOI":"10.1016\/j.spinee.2013.02.014","volume":"13","author":"SF Shakur","year":"2013","unstructured":"Shakur SF, Takagi I, Jacobsohn JA, Golden BM, Karahalios DG (2013) Spinal fibromatosis: a report of two cases and review of the literature. Spine J 13(8):e1\u2013e6","journal-title":"Spine J"},{"issue":"3","key":"481_CR39","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1159\/000345594","volume":"48","author":"B Wang","year":"2013","unstructured":"Wang B, Ma J, Jin H (2013) Infantile intracranial aggressive fibromatosis: report of two cases with a review of the literature. Pediatr NeuroSurg 48(3):181\u2013186","journal-title":"Pediatr NeuroSurg"},{"issue":"8","key":"481_CR40","doi-asserted-by":"publisher","first-page":"814","DOI":"10.1002\/1097-0347(200012)22:8<814::AID-HED11>3.0.CO;2-#","volume":"22","author":"A Hoos","year":"2000","unstructured":"Hoos A, Lewis JJ, Urist MJ et al (2000) Desmoid tumors of the head and neck\u2014a clinical study of a rare entity. Head Neck: J Sci Specialties Head Neck 22(8):814\u2013821","journal-title":"Head Neck: J Sci Specialties Head Neck"},{"issue":"6","key":"481_CR41","doi-asserted-by":"publisher","first-page":"897","DOI":"10.1007\/s10350-008-9232-5","volume":"51","author":"J Church","year":"2008","unstructured":"Church J, Lynch C, Neary P, LaGuardia L, Elayi E (2008) A desmoid tumor-staging system separates patients with intra-abdominal, Familial adenomatous polyposis-associated desmoid disease by behavior and prognosis. Diseases colon rectum 51(6):897\u2013901","journal-title":"Diseases colon rectum"},{"key":"481_CR42","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1023\/A:1013841813544","volume":"1","author":"AL Knudsen","year":"2001","unstructured":"Knudsen AL, B\u00fclow S (2001) Desmoid tumour in Familial adenomatous polyposis. A review of literature. Fam Cancer 1:113\u2013121","journal-title":"Fam Cancer"},{"issue":"6","key":"481_CR43","doi-asserted-by":"publisher","first-page":"926","DOI":"10.1080\/17453670610013240","volume":"77","author":"BM Dal\u00e9n","year":"2006","unstructured":"Dal\u00e9n BM, Meis-Kindblom JM, Sumathi VP, Ryd W, Kindblom LG (2006) Fine-needle aspiration cytology and core needle biopsy in the preoperative diagnosis of desmoid tumors. Acta Orthop 77(6):926\u2013931","journal-title":"Acta Orthop"},{"key":"481_CR44","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1007\/s10689-005-5675-1","volume":"5","author":"NJH Sturt","year":"2006","unstructured":"Sturt NJH, Clark SK (2006) Current ideas in desmoid tumours. Fam Cancer 5:275\u2013285","journal-title":"Fam Cancer"},{"issue":"9","key":"481_CR45","doi-asserted-by":"publisher","first-page":"1185","DOI":"10.1046\/j.1365-2168.1999.01222.x","volume":"86","author":"S Clark","year":"1999","unstructured":"Clark S, Neale K, Landgrebe J, Phillips R (1999) Desmoid tumours complicating Familial adenomatous polyposis. J Br Surg 86(9):1185\u20131189","journal-title":"J Br Surg"},{"issue":"6","key":"481_CR46","doi-asserted-by":"publisher","first-page":"737","DOI":"10.1016\/j.dld.2022.03.004","volume":"54","author":"N Benech","year":"2022","unstructured":"Benech N, Bonvalot S, Dufresne A et al (2022) Desmoid tumors located in the abdomen or associated with adenomatous polyposis: French intergroup clinical practice guidelines for diagnosis, treatment, and follow-up (SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO, ACHBT, SFR). Dig Liver Disease 54(6):737\u2013746","journal-title":"Dig Liver Disease"},{"issue":"4","key":"481_CR47","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1001\/jamaoncol.2018.6449","volume":"5","author":"A Gronchi","year":"2019","unstructured":"Gronchi A, Jones RL (2019) Treatment of desmoid tumors in 2019. JAMA Oncol 5(4):567\u2013568","journal-title":"JAMA Oncol"},{"key":"481_CR48","doi-asserted-by":"publisher","first-page":"4096","DOI":"10.1245\/s10434-013-3197-x","volume":"20","author":"S Bonvalot","year":"2013","unstructured":"Bonvalot S, Tern\u00e8s N, Fiore M et al (2013) Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol 20:4096\u20134102","journal-title":"Ann Surg Oncol"},{"key":"481_CR49","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1016\/j.ejca.2017.06.017","volume":"83","author":"N Penel","year":"2017","unstructured":"Penel N, Le Cesne A, Bonvalot S et al (2017) Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French sarcoma group. Eur J Cancer 83:125\u2013131","journal-title":"Eur J Cancer"},{"key":"481_CR50","doi-asserted-by":"publisher","first-page":"2587","DOI":"10.1245\/s10434-009-0586-2","volume":"16","author":"M Fiore","year":"2009","unstructured":"Fiore M, Rimareix F, Mariani L et al (2009) Desmoid-type fibromatosis: a front-line Conservative approach to select patients for surgical treatment. Ann Surg Oncol 16:2587\u20132593","journal-title":"Ann Surg Oncol"},{"key":"481_CR51","doi-asserted-by":"publisher","first-page":"2817","DOI":"10.1245\/s10434-015-4632-y","volume":"22","author":"DL van Broekhoven","year":"2015","unstructured":"van Broekhoven DL, Gr\u00fcnhagen DJ, den Bakker MA, van Dalen T, Verhoef C (2015) Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study. Ann Surg Oncol 22:2817\u20132823","journal-title":"Ann Surg Oncol"},{"issue":"18","key":"481_CR52","doi-asserted-by":"publisher","first-page":"4092","DOI":"10.1158\/1078-0432.CCR-21-4504","volume":"28","author":"MJ Nathenson","year":"2022","unstructured":"Nathenson MJ, Hu J, Ratan R et al (2022) Systemic chemotherapies retain antitumor activity in desmoid tumors independent of specific mutations in CTNNB1 or APC: a multi-institutional retrospective study. Clin Cancer Res 28(18):4092\u20134104","journal-title":"Clin Cancer Res"},{"issue":"25","key":"481_CR53","doi-asserted-by":"publisher","first-page":"2417","DOI":"10.1056\/NEJMoa1805052","volume":"379","author":"MM Gounder","year":"2018","unstructured":"Gounder MM, Mahoney MR, Van Tine BA et al (2018) Sorafenib for advanced and refractory desmoid tumors. N Engl J Med 379(25):2417\u20132428","journal-title":"N Engl J Med"},{"issue":"9","key":"481_CR54","doi-asserted-by":"publisher","first-page":"1263","DOI":"10.1016\/S1470-2045(19)30276-1","volume":"20","author":"M Toulmonde","year":"2019","unstructured":"Toulmonde M, Pulido M, Ray-Coquard I et al (2019) Pazopanib or methotrexate\u2013vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol 20(9):1263\u20131272","journal-title":"Lancet Oncol"},{"issue":"10","key":"481_CR55","doi-asserted-by":"publisher","first-page":"898","DOI":"10.1056\/NEJMoa2210140","volume":"388","author":"M Gounder","year":"2023","unstructured":"Gounder M, Ratan R, Alcindor T et al (2023) Nirogacestat, a \u03b3-secretase inhibitor for desmoid tumors. N Engl J Med 388(10):898\u2013912","journal-title":"N Engl J Med"},{"key":"481_CR56","doi-asserted-by":"crossref","unstructured":"Kalady MF, Church JM (2011) Monitoring and management of desmoids and other extracolonic manifestations in Familial adenomatous polyposis, vol 22. Elsevier, pp 112\u2013117","DOI":"10.1053\/j.scrs.2010.12.011"},{"issue":"6","key":"481_CR57","doi-asserted-by":"publisher","first-page":"853","DOI":"10.1007\/s10147-024-02506-2","volume":"29","author":"CR Oh","year":"2024","unstructured":"Oh CR, Jeong H, Kim W et al (2024) Favorable clinical efficacy of cytotoxic chemotherapy in patients with progressive desmoid tumors: a retrospective real-world study. Int J Clin Oncol 29(6):853\u2013861","journal-title":"Int J Clin Oncol"},{"issue":"12","key":"481_CR58","doi-asserted-by":"publisher","first-page":"1943","DOI":"10.1007\/s11605-010-1288-6","volume":"14","author":"FG de Campos","year":"2010","unstructured":"de Campos FG, Perez RO, Imperiale AR, Seid VE, Nahas SC, Cecconello I (2010) Evaluating causes of death in Familial adenomatous polyposis. J Gastrointest Surg 14(12):1943\u20131949","journal-title":"J Gastrointest Surg"},{"issue":"3","key":"481_CR59","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1093\/hmg\/4.3.337","volume":"4","author":"R Caspari","year":"1995","unstructured":"Caspari R, Olschwang S, Friedl W et al (1995) Familial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444. Hum Mol Genet 4(3):337\u2013340","journal-title":"Hum Mol Genet"},{"issue":"12","key":"481_CR60","doi-asserted-by":"publisher","first-page":"1832","DOI":"10.1136\/gut.2004.042705","volume":"53","author":"N Sturt","year":"2004","unstructured":"Sturt N, Gallagher M, Bassett P et al (2004) Evidence for genetic predisposition to desmoid tumours in Familial adenomatous polyposis independent of the germline APC mutation. Gut 53(12):1832\u20131836","journal-title":"Gut"},{"issue":"4","key":"481_CR61","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1097\/DCR.0000000000000316","volume":"58","author":"J Church","year":"2015","unstructured":"Church J, Xhaja X, LaGuardia L, O\u2019Malley M, Burke C, Kalady M (2015) Desmoids and genotype in Familial adenomatous polyposis. Diseases Colon Rectum 58(4):444\u2013448","journal-title":"Diseases Colon Rectum"},{"issue":"2","key":"481_CR62","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1002\/humu.1380030206","volume":"3","author":"ML Bisgaard","year":"1994","unstructured":"Bisgaard ML, Fenger K, B\u00fclow S, Niebuhr E, Mohr J (1994) Familial adenomatous polyposis (FAP): frequency, penetrance, and mutation rate. Hum Mutat 3(2):121\u2013125","journal-title":"Hum Mutat"},{"issue":"11","key":"481_CR63","doi-asserted-by":"publisher","first-page":"1351","DOI":"10.1097\/DCR.0000000000002172","volume":"65","author":"J Sommovilla","year":"2022","unstructured":"Sommovilla J, Liska D, Jia X et al (2022) IPAA is more desmoidogenic than ileorectal anastomosis in Familial adenomatous polyposis. Diseases Colon Rectum 65(11):1351\u20131361","journal-title":"Diseases Colon Rectum"},{"issue":"5","key":"481_CR64","doi-asserted-by":"publisher","first-page":"558","DOI":"10.1002\/bjs.9411","volume":"101","author":"M Vitellaro","year":"2014","unstructured":"Vitellaro M, Sala P, Signoroni S et al (2014) Risk of desmoid tumours after open and laparoscopic colectomy in patients with Familial adenomatous polyposis. J Br Surg 101(5):558\u2013565","journal-title":"J Br Surg"},{"issue":"2","key":"481_CR65","doi-asserted-by":"publisher","first-page":"e1571","DOI":"10.1097\/TXD.0000000000001571","volume":"10","author":"E Canovai","year":"2024","unstructured":"Canovai E, Butler A, Clark S et al (2024) Treatment of complex desmoid tumors in Familial adenomatous polyposis syndrome by intestinal transplantation. Transplantation Direct 10(2):e1571","journal-title":"Transplantation Direct"},{"issue":"3","key":"481_CR66","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1067\/msy.2001.110770","volume":"129","author":"MA Chatzipetrou","year":"2001","unstructured":"Chatzipetrou MA, Tzakis AG, Pinna AD et al (2001) Intestinal transplantation for the treatment of desmoid tumors associated with Familial adenomatous polyposis. Surgery 129(3):277\u2013281","journal-title":"Surgery"},{"issue":"4","key":"481_CR67","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1002\/jso.20416","volume":"92","author":"JI Moon","year":"2005","unstructured":"Moon JI, Selvaggi G, Nishida S et al (2005) Intestinal transplantation for the treatment of neoplastic disease. J Surg Oncol 92(4):284\u2013291","journal-title":"J Surg Oncol"}],"container-title":["Familial Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10689-025-00481-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10689-025-00481-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10689-025-00481-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,19]],"date-time":"2025-09-19T05:03:18Z","timestamp":1758258198000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10689-025-00481-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,20]]},"references-count":67,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,9]]}},"alternative-id":["481"],"URL":"https:\/\/doi.org\/10.1007\/s10689-025-00481-9","relation":{},"ISSN":["1573-7292"],"issn-type":[{"value":"1573-7292","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,20]]},"assertion":[{"value":"16 February 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 May 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 June 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"56"}}