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Fibromatosis

MedGen UID:
8836
•Concept ID:
C0016048
•
Neoplastic Process
Synonym: Fibromatoses
SNOMED CT: Fibromatosis (19928005); Fibromatosis (723976005)
 
Monarch Initiative: MONDO:0005031

Definition

A poorly circumscribed neoplasm arising from the soft tissues. It is characterized by the presence of spindle-shaped fibroblasts and an infiltrative growth pattern. [from NCI]

Professional guidelines

PubMed

Evolving strategies for management of desmoid tumor.
Riedel RF, Agulnik M
Cancer 2022 Aug 15;128(16):3027-3040. Epub 2022 Jun 7 doi: 10.1002/cncr.34332. PMID: 35670122Free PMC Article
The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.
Desmoid Tumor Working Group
Eur J Cancer 2020 Mar;127:96-107. Epub 2020 Jan 28 doi: 10.1016/j.ejca.2019.11.013. PMID: 32004793
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).
Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, Hohenberger P, Penel N, Messiou C, van der Graaf WT, Gronchi A; Desmoid Working Group
Ann Oncol 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323. PMID: 28961825Free PMC Article

Recent clinical studies

Etiology

Desmoid Tumors: Current Perspective and Treatment.
Mangla A, Agarwal N, Schwartz G
Curr Treat Options Oncol 2024 Feb;25(2):161-175. Epub 2024 Jan 25 doi: 10.1007/s11864-024-01177-5. PMID: 38270798Free PMC Article
Desmoid Tumors: A Comprehensive Review.
Bektas M, Bell T, Khan S, Tumminello B, Fernandez MM, Heyes C, Oton AB
Adv Ther 2023 Sep;40(9):3697-3722. Epub 2023 Jul 12 doi: 10.1007/s12325-023-02592-0. PMID: 37436594Free PMC Article
Evolving strategies for management of desmoid tumor.
Riedel RF, Agulnik M
Cancer 2022 Aug 15;128(16):3027-3040. Epub 2022 Jun 7 doi: 10.1002/cncr.34332. PMID: 35670122Free PMC Article
The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.
Desmoid Tumor Working Group
Eur J Cancer 2020 Mar;127:96-107. Epub 2020 Jan 28 doi: 10.1016/j.ejca.2019.11.013. PMID: 32004793
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).
Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, Hohenberger P, Penel N, Messiou C, van der Graaf WT, Gronchi A; Desmoid Working Group
Ann Oncol 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323. PMID: 28961825Free PMC Article

Diagnosis

Desmoid Tumors: Current Perspective and Treatment.
Mangla A, Agarwal N, Schwartz G
Curr Treat Options Oncol 2024 Feb;25(2):161-175. Epub 2024 Jan 25 doi: 10.1007/s11864-024-01177-5. PMID: 38270798Free PMC Article
Desmoid Tumors: A Comprehensive Review.
Bektas M, Bell T, Khan S, Tumminello B, Fernandez MM, Heyes C, Oton AB
Adv Ther 2023 Sep;40(9):3697-3722. Epub 2023 Jul 12 doi: 10.1007/s12325-023-02592-0. PMID: 37436594Free PMC Article
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).
Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, Hohenberger P, Penel N, Messiou C, van der Graaf WT, Gronchi A; Desmoid Working Group
Ann Oncol 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323. PMID: 28961825Free PMC Article
Mesenteric fibromatosis.
Chaudhary P
Int J Colorectal Dis 2014 Dec;29(12):1445-51. Epub 2014 Aug 21 doi: 10.1007/s00384-014-1995-7. PMID: 25139370
Aggressive fibromatosis.
Fisher C, Thway K
Pathology 2014 Feb;46(2):135-40. doi: 10.1097/PAT.0000000000000045. PMID: 24378386

Therapy

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
Nirogacestat, a γ-Secretase Inhibitor for Desmoid Tumors.
Gounder M, Ratan R, Alcindor T, Schöffski P, van der Graaf WT, Wilky BA, Riedel RF, Lim A, Smith LM, Moody S, Attia S, Chawla S, D'Amato G, Federman N, Merriam P, Van Tine BA, Vincenzi B, Benson C, Bui NQ, Chugh R, Tinoco G, Charlson J, Dileo P, Hartner L, Lapeire L, Mazzeo F, Palmerini E, Reichardt P, Stacchiotti S, Bailey HH, Burgess MA, Cote GM, Davis LE, Deshpande H, Gelderblom H, Grignani G, Loggers E, Philip T, Pressey JG, Kummar S, Kasper B
N Engl J Med 2023 Mar 9;388(10):898-912. doi: 10.1056/NEJMoa2210140. PMID: 36884323Free PMC Article
Sorafenib for Advanced and Refractory Desmoid Tumors.
Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ, Riedel RF, Sabagh T, Tap WD, Horvat N, Basch E, Schwartz LH, Maki RG, Agaram NP, Lefkowitz RA, Mazaheri Y, Yamashita R, Wright JJ, Dueck AC, Schwartz GK
N Engl J Med 2018 Dec 20;379(25):2417-2428. doi: 10.1056/NEJMoa1805052. PMID: 30575484Free PMC Article
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).
Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, Hohenberger P, Penel N, Messiou C, van der Graaf WT, Gronchi A; Desmoid Working Group
Ann Oncol 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323. PMID: 28961825Free PMC Article
Plantar fibromatosis--topical review.
Veith NT, Tschernig T, Histing T, Madry H
Foot Ankle Int 2013 Dec;34(12):1742-6. Epub 2013 Sep 16 doi: 10.1177/1071100713505535. PMID: 24043350

Prognosis

Desmoid-type fibromatosis: imaging features and course.
Okuda M, Yoshida K, Kobayashi S, Gabata T
Skeletal Radiol 2023 Jul;52(7):1293-1303. Epub 2023 Jan 16 doi: 10.1007/s00256-023-04275-x. PMID: 36646850
Evolving strategies for management of desmoid tumor.
Riedel RF, Agulnik M
Cancer 2022 Aug 15;128(16):3027-3040. Epub 2022 Jun 7 doi: 10.1002/cncr.34332. PMID: 35670122Free PMC Article
The Landmark Series: Desmoid.
Fiore M, Crago A, Gladdy R, Kasper B
Ann Surg Oncol 2021 Mar;28(3):1682-1689. Epub 2021 Jan 1 doi: 10.1245/s10434-020-09395-5. PMID: 33386543
Sorafenib for Advanced and Refractory Desmoid Tumors.
Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ, Riedel RF, Sabagh T, Tap WD, Horvat N, Basch E, Schwartz LH, Maki RG, Agaram NP, Lefkowitz RA, Mazaheri Y, Yamashita R, Wright JJ, Dueck AC, Schwartz GK
N Engl J Med 2018 Dec 20;379(25):2417-2428. doi: 10.1056/NEJMoa1805052. PMID: 30575484Free PMC Article
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).
Kasper B, Baumgarten C, Garcia J, Bonvalot S, Haas R, Haller F, Hohenberger P, Penel N, Messiou C, van der Graaf WT, Gronchi A; Desmoid Working Group
Ann Oncol 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323. PMID: 28961825Free PMC Article

Clinical prediction guides

Current Management of Desmoid Tumors: A Review.
Kasper B, Baldini EH, Bonvalot S, Callegaro D, Cardona K, Colombo C, Corradini N, Crago AM, Dei Tos AP, Dileo P, Elnekave E, Erinjeri JP, Navid F, Farma JM, Ferrari A, Fiore M, Gladdy RA, Gounder M, Haas RL, Husson O, Kurtz JE, Lazar AJ, Orbach D, Penel N, Ratan R, Raut CP, Roland CL, Schut AW, Sparber-Sauer M, Strauss DC, Van der Graaf WTA, Vitellaro M, Weiss AR, Gronchi A; Desmoid Tumor Working Group
JAMA Oncol 2024 Aug 1;10(8):1121-1128. doi: 10.1001/jamaoncol.2024.1805. PMID: 38900421
Desmoid Tumors: Current Perspective and Treatment.
Mangla A, Agarwal N, Schwartz G
Curr Treat Options Oncol 2024 Feb;25(2):161-175. Epub 2024 Jan 25 doi: 10.1007/s11864-024-01177-5. PMID: 38270798Free PMC Article
The Landmark Series: Desmoid.
Fiore M, Crago A, Gladdy R, Kasper B
Ann Surg Oncol 2021 Mar;28(3):1682-1689. Epub 2021 Jan 1 doi: 10.1245/s10434-020-09395-5. PMID: 33386543
Locally Aggressive Connective Tissue Tumors.
Gounder MM, Thomas DM, Tap WD
J Clin Oncol 2018 Jan 10;36(2):202-209. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.8482. PMID: 29220303Free PMC Article
Management of Desmoids.
Grignol VP, Pollock R, Howard JH
Surg Clin North Am 2016 Oct;96(5):1015-30. doi: 10.1016/j.suc.2016.05.008. PMID: 27542640

Recent systematic reviews

Colectomy and desmoid tumours in familial adenomatous polyposis: a systematic review and meta-analysis.
Aelvoet AS, Struik D, Bastiaansen BAJ, Bemelman WA, Hompes R, Bossuyt PMM, Dekker E
Fam Cancer 2022 Oct;21(4):429-439. Epub 2022 Jan 13 doi: 10.1007/s10689-022-00288-y. PMID: 35022961Free PMC Article
Pachydermodactyly: a systematic review.
Vázquez Fernández R, Maneiro Fernández JR, Cervantes Pérez EC, Mera Varela A
Ir J Med Sci 2021 Aug;190(3):1005-1014. Epub 2020 Oct 1 doi: 10.1007/s11845-020-02378-1. PMID: 33006046
Syndromes with gingival fibromatosis: A systematic review.
Costa CRR, Braz SV, de Toledo IP, Martelli-Júnior H, Mazzeu JF, Guerra ENS, Coletta RD, Acevedo AC
Oral Dis 2021 May;27(4):881-893. Epub 2020 May 25 doi: 10.1111/odi.13369. PMID: 32335995
Reconstruction of Desmoid Tumors: Case Series and Systematic Review.
Ver Halen JP, Soto-Miranda MA, Sandoval JA
Ann Plast Surg 2015 Oct;75(4):480-6. doi: 10.1097/SAP.0000000000000129. PMID: 26360656
Hereditary gingival fibromatosis: a systematic review.
Coletta RD, Graner E
J Periodontol 2006 May;77(5):753-64. doi: 10.1902/jop.2006.050379. PMID: 16671866

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