Advertisement

Management of Desmoid Tumors

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribers receive full online access to your subscription and archive of back issues up to and including 2002.

      Content published before 2002 is available via pay-per-view purchase only.

      Subscribe:

      Subscribe to Surgical Oncology Clinics
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Fiore M.
        • Crago A.
        • Gladdy R.
        • et al.
        The landmark series: desmoid.
        Ann Surg Oncol. 2021; 28: 1682-1689
        • Kasper B.
        • Baumgarten C.
        • Garcia J.
        • et al.
        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. 2017; 28: 2399-2408
        • Cuomo P.
        • Scoccianti G.
        • Schiavo A.
        • et al.
        Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.
        BMC Cancer. 2021; 21: 437
      1. Board. WCoTE. Soft tissue and bone tumours. 5th edition ed. Lyon2020.

        • Fiore M.
        • Coppola S.
        • Cannell A.J.
        • et al.
        Desmoid-type fibromatosis and pregnancy: a multi-institutional analysis of recurrence and obstetric risk.
        Ann Surg. 2014; 259: 973-978
        • Cojocaru E.
        • Gennatas S.
        • Thway K.
        • et al.
        Approach to screening for familial adenomatous polyposis (FAP) in a cohort of 226 patients with desmoid-type fibromatosis (DF): experience of a specialist center in the UK.
        Fam Cancer. 2021; 1: 69-74
        • Gronchi A.
        • Jones R.L.
        Treatment of desmoid tumors in 2019.
        JAMA Oncol. 2019; 5: 567-568
        • Kasper B.
        • Raut C.P.
        • Gronchi A.
        Desmoid tumors: to treat or not to treat, that is the question.
        Cancer. 2020; 126: 5213-5221
      2. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients.
        Eur J Cancer. 2020; 127: 96-107
        • Kummar S.
        • O'Sullivan Coyne G.
        • Do K.T.
        • et al.
        Clinical activity of the γ-secretase inhibitor PF-03084014 in adults with desmoid tumors (aggressive fibromatosis).
        J Clin Oncol. 2017; 35: 1561-1569
        • Crago A.M.
        • Chmielecki J.
        • Rosenberg M.
        • et al.
        Near universal detection of alterations in CTNNB1 and Wnt pathway regulators in desmoid-type fibromatosis by whole-exome sequencing and genomic analysis.
        Genes Chromosomes Cancer. 2015; 54: 606-615
        • Slowik V.
        • Attard T.
        • Dai H.
        • et al.
        Desmoid tumors complicating familial adenomatous polyposis: a meta-analysis mutation spectrum of affected individuals.
        BMC Gastroenterol. 2015; 15: 84
        • Timbergen M.J.M.
        • Colombo C.
        • Renckens M.
        • et al.
        The prognostic role of β-catenin mutations in desmoid-type fibromatosis undergoing resection only: a meta-analysis of individual patient data.
        Ann Surg. 2021; 273: 1094-1101
        • Hamada S.
        • Futamura N.
        • Ikuta K.
        • et al.
        CTNNB1 S45F mutation predicts poor efficacy of meloxicam treatment for desmoid tumors: a pilot study.
        PLoS One. 2014; 9e96391
        • Kasper B.
        • Gruenwald V.
        • Reichardt P.
        • et al.
        Correlation of CTNNB1 mutation status with progression arrest rate in RECIST progressive desmoid-type fibromatosis treated with imatinib: translational research results from a phase 2 study of the German Interdisciplinary Sarcoma Group (GISG-01).
        Ann Surg Oncol. 2016; 23: 1924-1927
        • Timbergen M.J.M.
        • Boers R.
        • Vriends A.L.M.
        • et al.
        Differentially methylated regions in desmoid-type fibromatosis: a comparison between CTNNB1 S45F and T41A tumors.
        Front Oncol. 2020; 10: 565031
        • Bräutigam K.
        • Lindner J.
        • Budczies J.
        • et al.
        PARP-1 expression as a prognostic factor in desmoid-type fibromatosis.
        Ann Diagn Pathol. 2020; 44: 151442
        • Colombo C.
        • Fiore M.
        • Grignani G.
        • et al.
        A prospective observational study of active surveillance in primary desmoid fibromatosis.
        Clin Cancer Res. 2022; (clincanres.4205)https://doi.org/10.1158/1078-0432.CCR-21-4205
        • Bonvalot S.
        • Ternès N.
        • Fiore M.
        • et al.
        Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought.
        Ann Surg Oncol. 2013; 20: 4096-4102
        • Fiore M.
        • Rimareix F.
        • Mariani L.
        • et al.
        Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment.
        Ann Surg Oncol. 2009; 16: 2587-2593
        • Penel N.
        • Le Cesne A.
        • Bonvalot S.
        • et al.
        Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French Sarcoma Group.
        Eur J Cancer. 2017; 83: 125-131
        • Ruspi L.
        • Cananzi F.C.M.
        • Sicoli F.
        • et al.
        Event-free survival in desmoid-type fibromatosis (DTF): a pre-post comparison of upfront surgery versus wait-and-see approach.
        Eur J Surg Oncol. 2021; 47: 1196-1200
      3. Quality of life of patients with desmoid-type fibromatosis (QUALIFIED) [updated April 2, 2021 August 31, 2021]. ClinicalTrials.gov Identifier: NCT04289077].
        (Available at:)
        • Timbergen M.J.M.
        • Schut A.W.
        • Grünhagen D.J.
        • et al.
        Active surveillance in desmoid-type fibromatosis: a systematic literature review.
        Eur J Cancer. 2020; 137: 18-29
        • Colombo C.
        • Miceli R.
        • Le Péchoux C.
        • et al.
        Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients.
        Eur J Cancer. 2015; 51: 186-192
        • Improta L.
        • Tzanis D.
        • Bouhadiba T.
        • et al.
        Desmoid tumours in the surveillance era: what are the remaining indications for surgery?.
        Eur J Surg Oncol. 2020; 46: 1310-1314
        • Sanchez-Mete L.
        • Ferraresi V.
        • Caterino M.
        • et al.
        Desmoid tumors characteristics, clinical management, active surveillance, and description of our FAP case series.
        J Clin Med. 2020; 9
        • Duhil de Bénazé G.
        • Vigan M.
        • Corradini N.
        • et al.
        Functional analysis of young patients with desmoid-type fibromatosis: Initial surveillance does not jeopardize long term quality of life.
        Eur J Surg Oncol. 2020; 46: 1294-1300
        • Gronchi A.
        • Casali P.G.
        • Mariani L.
        • et al.
        Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution.
        J Clin Oncol. 2003; 21: 1390-1397
        • Crago A.M.
        • Denton B.
        • Salas S.
        • et al.
        A prognostic nomogram for prediction of recurrence in desmoid fibromatosis.
        Ann Surg. 2013; 258: 347-353
      4. Braggio DL, A.; Hernandez, L.; Mercier, K. A patient reported outcomes of treatments for desmoid tumors: an international natural history study. 2021 ASCO Annual Meeting.

        • Bouttefroy SP N.
        • Minard-Colin V.
        • Orbach D.
        • et al.
        Desmoid type fibromatosis in patients.
        ESMO Virtual Congress. 2020;
      5. National clinical-biological prospective cohort of incident cases of aggressive fibromatosis (ALTITUDES) [updated May 13, 2021. ClinicalTrials.gov Identifier: NCT02867033].
        (Available at:)
        • Bishop A.J.
        • Landry J.P.
        • Roland C.L.
        • et al.
        Certain risk factors for patients with desmoid tumors warrant reconsideration of local therapy strategies.
        Cancer. 2020; 126: 3265-3273
        • Hansmann A.
        • Adolph C.
        • Vogel T.
        • et al.
        High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors.
        Cancer. 2004; 100: 612-620
        • Skapek S.X.
        • Anderson J.R.
        • Hill D.A.
        • et al.
        Safety and efficacy of high-dose tamoxifen and sulindac for desmoid tumor in children: results of a Children's Oncology Group (COG) phase II study.
        Pediatr Blood Cancer. 2013; 60: 1108-1112
        • Fiore M.
        • Colombo C.
        • Radaelli S.
        • et al.
        Hormonal manipulation with toremifene in sporadic desmoid-type fibromatosis.
        Eur J Cancer. 2015; 51: 2800-2807
        • Quast D.R.
        • Schneider R.
        • Burdzik E.
        • et al.
        Long-term outcome of sporadic and FAP-associated desmoid tumors treated with high-dose selective estrogen receptor modulators and sulindac: a single-center long-term observational study in 134 patients.
        Fam Cancer. 2016; 15: 31-40
        • Azzarelli A.
        • Gronchi A.
        • Bertulli R.
        • et al.
        Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fibromatosis.
        Cancer. 2001; 92: 1259-1264
        • Gega M.
        • Yanagi H.
        • Yoshikawa R.
        • et al.
        Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis.
        J Clin Oncol. 2006; 24: 102-105
        • Heinrich M.C.
        • McArthur G.A.
        • Demetri G.D.
        • et al.
        Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor).
        J Clin Oncol. 2006; 24: 1195-1203
        • Chugh R.
        • Wathen J.K.
        • Patel S.R.
        • et al.
        Efficacy of imatinib in aggressive fibromatosis: results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial.
        Clin Cancer Res. 2010; 16: 4884-4891
        • Palassini E.
        • Frezza A.M.
        • Mariani L.
        • et al.
        Long-term efficacy of methotrexate plus vinblastine/vinorelbine in a large series of patients affected by desmoid-type fibromatosis.
        Cancer J. 2017; 23: 86-91
        • Gounder M.M.
        • Mahoney M.R.
        • Van Tine B.A.
        • et al.
        Sorafenib for advanced and refractory desmoid tumors.
        N Engl J Med. 2018; 379: 2417-2428
        • Toulmonde M.
        • Pulido M.
        • Ray-Coquard I.
        • et al.
        Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study.
        Lancet Oncol. 2019; 20: 1263-1272
        • Gounder M.M.
        • Lefkowitz R.A.
        • Keohan M.L.
        • et al.
        Activity of sorafenib against desmoid tumor/deep fibromatosis.
        Clin Cancer Res. 2011; 17: 4082-4090
        • Kasper B.
        • Gruenwald V.
        • Reichardt P.
        • et al.
        Imatinib induces sustained progression arrest in RECIST progressive desmoid tumours: final results of a phase II study of the German Interdisciplinary Sarcoma Group (GISG).
        Eur J Cancer. 2017; 76: 60-67
        • Szucs Z.
        • Messiou C.
        • Wong H.H.
        • et al.
        Pazopanib, a promising option for the treatment of aggressive fibromatosis.
        Anticancer Drugs. 2017; 28: 421-426
        • Zheng C.
        • Zhou Y.
        • Wang Y.
        • et al.
        The activity and safety of anlotinib for patients with extremity desmoid fibromatosis: a retrospective study in a single institution.
        Drug Des Devel Ther. 2020; 14: 3941-3950
        • Zheng C.
        • Fang J.
        • Wang Y.
        • et al.
        Efficacy and safety of apatinib for patients with advanced extremity desmoid fibromatosis: a retrospective study.
        J Cancer Res Clin Oncol. 2021; 147: 2127-2135
        • Nishida Y.
        • Hamada S.
        • Urakawa H.
        • et al.
        Desmoid with biweekly methotrexate and vinblastine shows similar effects to weekly administration: a phase II clinical trial.
        Cancer Sci. 2020; 111: 4187-4194
        • Napolitano A.
        • Provenzano S.
        • Colombo C.
        • et al.
        Familial adenomatosis polyposis-related desmoid tumours treated with low-dose chemotherapy: results from an international, multi-institutional, retrospective analysis.
        ESMO Open. 2020; 5
        • Gennatas S.
        • Chamberlain F.
        • Smrke A.
        • et al.
        A timely oral option: single-agent vinorelbine in desmoid tumors.
        Oncologist. 2020; 25: e2013-e2016
        • Constantinidou A.
        • Jones R.L.
        • Scurr M.
        • et al.
        Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis.
        Eur J Cancer. 2009; 45: 2930-2934
        • Garbay D.
        • Le Cesne A.
        • Penel N.
        • et al.
        Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG).
        Ann Oncol. 2012; 23: 182-186
        • Shimizu K.
        • Kawashima H.
        • Kawai A.
        • et al.
        Effectiveness of doxorubicin-based and liposomal doxorubicin chemotherapies for patients with extra-abdominal desmoid-type fibromatosis: a systematic review.
        Jpn J Clin Oncol. 2020; 50: 1274-1281
        • Gounder M.M.
        Notch inhibition in desmoids: "sure it works in practice, but does it work in theory?.
        Cancer. 2015; 121: 3933-3937
        • Messersmith W.A.
        • Shapiro G.I.
        • Cleary J.M.
        • et al.
        A phase I, dose-finding study in patients with advanced solid malignancies of the oral γ-secretase inhibitor PF-03084014.
        Clin Cancer Res. 2015; 21: 60-67
      6. Nirogacestat for adults with desmoid tumor/aggressive fibromatosis (DT/AF) (DeFi) [updated July 27, 2021August 31, 2021]. ClinicalTrials.gov Identifier: NCT03785964].
        (Available at:)
      7. A study of AL102 in patients with progressing desmoid tumors (RINGSIDE). 2021 (ClinicalTrials.gov Identifier: NCT04871282]. Available at:)
        • Keus R.B.
        • Nout R.A.
        • Blay J.Y.
        • et al.
        Results of a phase II pilot study of moderate dose radiotherapy for inoperable desmoid-type fibromatosis--an EORTC STBSG and ROG study (EORTC 62991-22998).
        Ann Oncol. 2013; 24: 2672-2676
        • Gladdy R.A.
        • Qin L.X.
        • Moraco N.
        • et al.
        Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas?.
        J Clin Oncol. 2010; 28: 2064-2069
        • Bouhamama A.
        • Lame F.
        • Mastier C.
        • et al.
        Local control and analgesic efficacy of percutaneous cryoablation for desmoid tumors.
        Cardiovasc Intervent Radiol. 2020; 43: 110-119
        • Saltiel S.
        • Bize P.E.
        • Goetti P.
        • et al.
        Cryoablation of extra-abdominal desmoid tumors: a single-center experience with literature review.
        Diagnostics (Basel). 2020; 10
        • Kurtz J.E.
        • Buy X.
        • Deschamps F.
        • et al.
        CRYODESMO-O1: a prospective, open phase II study of cryoablation in desmoid tumour patients progressing after medical treatment.
        Eur J Cancer. 2021; 143: 78-87
        • Lewis J.J.
        • Boland P.J.
        • Leung D.H.
        • et al.
        The enigma of desmoid tumors.
        Ann Surg. 1999; 229: 866-872
        • Burtenshaw S.M.
        • Cannell A.J.
        • McAlister E.D.
        • et al.
        Toward observation as first-line management in abdominal desmoid tumors.
        Ann Surg Oncol. 2016; 23: 2212-2219
        • Cassidy M.R.
        • Lefkowitz R.A.
        • Long N.
        • et al.
        Association of MRI T2 signal intensity with desmoid tumor progression during active observation: a retrospective cohort study.
        Ann Surg. 2020; 271: 748-755
        • Murahashi Y.
        • Emori M.
        • Shimizu J.
        • et al.
        The value of the black fiber sign on T1-weighted images for predicting stability of desmoid fibromatosis managed conservatively.
        Eur Radiol. 2020; 30: 5768-5776
        • Gondim Teixeira P.A.
        • Biouichi H.
        • Abou Arab W.
        • et al.
        Evidence-based MR imaging follow-up strategy for desmoid-type fibromatosis.
        Eur Radiol. 2020; 30: 895-902
        • Crombé A.
        • Kind M.
        • Ray-Coquard I.
        • et al.
        Progressive desmoid tumor: radiomics compared with conventional response criteria for predicting progression during systemic therapy-a multicenter study by the French Sarcoma Group.
        AJR Am J Roentgenol. 2020; 215: 1539-1548
        • Timbergen M.J.M.
        • Starmans M.P.A.
        • Padmos G.A.
        • et al.
        Differential diagnosis and mutation stratification of desmoid-type fibromatosis on MRI using radiomics.
        Eur J Radiol. 2020; 131: 109266
        • Newman E.T.
        • Lans J.
        • Kim J.
        • et al.
        PROMIS function scores are lower in patients who underwent more aggressive local treatment for desmoid tumors.
        Clin Orthop Relat Res. 2020; 478: 563-577
        • Gounder M.M.
        • Maddux L.
        • Paty J.
        • et al.
        Prospective development of a patient-reported outcomes instrument for desmoid tumors or aggressive fibromatosis.
        Cancer. 2020; 126: 531-539
      8. (Available at:)
      9. (Available at:)
      10. Quality of life of patients with desmoid-type fibromatosis.
        (QUALIFIED) Available at: