U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Desmoid tumor

MedGen UID:
38187
Concept ID:
C0079218
Neoplastic Process
Synonym: Aggressive fibromatosis
SNOMED CT: Desmoid-type fibromatosis (47284001); Desmoid tumor (47284001); Aggressive fibromatosis (725049005); Desmoid type fibromatosis (725049005); Desmoid tumor (725049005); Desmoid fibromatosis (725049005); Deep fibromatosis (725049005); Aggressive fibromatosis (47284001); Invasive fibroma (47284001); Desmoid (47284001)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related gene: APC
 
HPO: HP:6001034
Monarch Initiative: MONDO:0007608
Orphanet: ORPHA873

Definition

Desmoid tumors are rare, locally aggressive, fibroblastic soft-tissue tumors that are characterized by infiltrative growth and tendency to recur. Desmoid tumors do not metastasize but can arise in any part of the body [from HPO]

Professional guidelines

PubMed

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
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
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

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
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
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free PMC Article
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
Gill SS, Heuman DM, Mihas AA
J Clin Gastroenterol 2001 Oct;33(4):267-82. doi: 10.1097/00004836-200110000-00004. PMID: 11588539

Diagnosis

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
Eizuka M, Toya Y, Kosaka T, Oizumi T, Morishita T, Kasugai S, Yanai S, Sugai T, Matsumoto T
Intern Med 2023 Sep 15;62(18):2651-2654. Epub 2023 Feb 1 doi: 10.2169/internalmedicine.1101-22. PMID: 36725040Free PMC Article
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
Erickson LA, Chen B
Mayo Clin Proc 2022 Jan;97(1):193-195. doi: 10.1016/j.mayocp.2021.11.025. PMID: 34996557
Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A
ESMO Open 2021 Jun;6(3):100170. Epub 2021 Jun 2 doi: 10.1016/j.esmoop.2021.100170. PMID: 34090171Free PMC Article

Therapy

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
Attia S, Bolejack V, Ganjoo KN, George S, Agulnik M, Rushing D, Loggers ET, Livingston MB, Wright J, Chawla SP, Okuno SH, Reinke DK, Riedel RF, Davis LE, Ryan CW, Maki RG
Cancer Med 2023 Jan;12(2):1532-1539. Epub 2022 Aug 10 doi: 10.1002/cam4.5044. PMID: 35950293Free PMC Article
Davis LE, Bolejack V, Ryan CW, Ganjoo KN, Loggers ET, Chawla S, Agulnik M, Livingston MB, Reed D, Keedy V, Rushing D, Okuno S, Reinke DK, Riedel RF, Attia S, Mascarenhas L, Maki RG
J Clin Oncol 2019 Jun 1;37(16):1424-1431. Epub 2019 Apr 23 doi: 10.1200/JCO.18.02374. PMID: 31013172Free PMC Article
Lacayo EA, Glastonbury CM, Hoang JK, Magliocca KR, Hill KL, Hudgins PA
AJR Am J Roentgenol 2016 May;206(5):1068-72. Epub 2016 Feb 25 doi: 10.2214/AJR.15.14864. PMID: 26914791
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Prognosis

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
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
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
Fiore M, MacNeill A, Gronchi A, Colombo C
Surg Oncol Clin N Am 2016 Oct;25(4):803-26. Epub 2016 Aug 5 doi: 10.1016/j.soc.2016.05.010. PMID: 27591500
Gill SS, Heuman DM, Mihas AA
J Clin Gastroenterol 2001 Oct;33(4):267-82. doi: 10.1097/00004836-200110000-00004. PMID: 11588539

Clinical prediction guides

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
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
Pistioli E, Soulioti E, Kapetanakis EI, Michos TP, Tomos PI, Sidiropoulou T
Medicina (Kaunas) 2022 Dec 16;58(12) doi: 10.3390/medicina58121857. PMID: 36557059Free PMC Article
Davis LE, Bolejack V, Ryan CW, Ganjoo KN, Loggers ET, Chawla S, Agulnik M, Livingston MB, Reed D, Keedy V, Rushing D, Okuno S, Reinke DK, Riedel RF, Attia S, Mascarenhas L, Maki RG
J Clin Oncol 2019 Jun 1;37(16):1424-1431. Epub 2019 Apr 23 doi: 10.1200/JCO.18.02374. PMID: 31013172Free PMC Article
Fiore M, MacNeill A, Gronchi A, Colombo C
Surg Oncol Clin N Am 2016 Oct;25(4):803-26. Epub 2016 Aug 5 doi: 10.1016/j.soc.2016.05.010. PMID: 27591500

Recent systematic reviews

Loureiro FJA, Nogueira WR, Dutra MJ, Stelter GO, Schuch LF, Silveira FM, Wagner VP, Martins MD, Martins MAT
Oral Dis 2025 Jan;31(1):32-38. Epub 2024 Oct 7 doi: 10.1111/odi.15153. PMID: 39375868
Meade A, Wisecarver I, Cho MJ, Davis JJ, Duque S, Farewell JT, Zhang AY
Ann Plast Surg 2023 May 1;90(5S Suppl 2):S195-S202. Epub 2022 Dec 21 doi: 10.1097/SAP.0000000000003381. PMID: 36729103
Vora BMK, Munk PL, Somasundaram N, Ouellette HA, Mallinson PI, Sheikh A, Abdul Kadir H, Tan TJ, Yan YY
PLoS One 2021;16(12):e0261657. Epub 2021 Dec 23 doi: 10.1371/journal.pone.0261657. PMID: 34941915Free PMC Article
Emori M, Matsumoto Y, Murahashi Y, Yoshida M, Nishida Y
Nagoya J Med Sci 2021 Nov;83(4):673-681. doi: 10.18999/nagjms.83.4.673. PMID: 34916712Free PMC Article
Smith K, Desai J, Lazarakis S, Gyorki D
Ann Surg Oncol 2018 Jun;25(6):1544-1554. Epub 2018 Apr 11 doi: 10.1245/s10434-018-6408-7. PMID: 29644533

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...