Skip to main content

Advertisement

Log in

Desmoid-type fibromatosis: imaging features and course

  • Review Article
  • Published:
Skeletal Radiology Aims and scope Submit manuscript

Abstract

Desmoid-type fibromatosis (DF) is a soft tissue tumor characterized by infiltrative growth and a tendency toward local recurrence, while it exhibits self-limiting behavior and shows spontaneous regression. With its unpredictable behavior, a change in treatment strategies from initial surgery to nonsurgical management has been proposed, and active surveillance is currently widely chosen as the initial treatment strategy for DF. We reviewed the imaging features of DF regarding its clinical course, focusing on regression cases, postoperative cases, and imaging changes after systemic treatment.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12

Similar content being viewed by others

References

  1. Bansal A, Goyal S, Goyal A, Jana M. WHO classification of soft tissue tumours 2020: An update and simplified approach for radiologists. Eur J Radiol. 2021;143: 109937.

    PubMed  Google Scholar 

  2. Colombo C, Gronchi A. Desmoid-type fibromatosis: what works best? European journal of cancer (Oxford. England. 1990;2009(45 Suppl 1):466–7.

    Google Scholar 

  3. Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, Tirumani SH, Shinagare AB, Hornick JL, et al. Role of imaging in management of desmoid-type fibromatosis: a primer for radiologists. Radiographics. 2016;36(3):767–82.

    PubMed  Google Scholar 

  4. Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66(9):1369–74.

    CAS  PubMed  Google Scholar 

  5. Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF. The enigma of desmoid tumors. Ann Surg. 1999; 229(6):866–872; discussion 872–863.

  6. Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16(9):2587–93.

    PubMed  Google Scholar 

  7. Burtenshaw SM, Cannell AJ, McAlister ED, Siddique S, Kandel R, Blackstein ME, et al. Toward observation as first-line management in abdominal desmoid tumors. Ann Surg Oncol. 2016;23(7):2212–9.

    PubMed  Google Scholar 

  8. Peng PD, Hyder O, Mavros MN, Turley R, Groeschl R, Firoozmand A, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19(13):4036–42.

    PubMed  PubMed Central  Google Scholar 

  9. Nakayama T, Tsuboyama T, Toguchida J, Hosaka T, Nakamura T. Natural course of desmoid-type fibromatosis. J Orthop Sci. 2008;13(1):51–5.

    PubMed  Google Scholar 

  10. de Bruyns A, Li H, MacNeil A, Simmons C, Clarkson P, Goddard K, et al. Evolving practice patterns over two decades (1993–2013) in the management of desmoid-type fibromatosis in British Columbia. Clin Oncol (R Coll Radiol). 2020;32(4):e102–10.

    PubMed  Google Scholar 

  11. Crago AM, Denton B, Salas S, Dufresne A, Mezhir JJ, Hameed M, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. 2013;258(2):347–53.

    PubMed  Google Scholar 

  12. Shido Y, Nishida Y, Nakashima H, Katagiri H, Sugiura H, Yamada Y, et al. Surgical treatment for local control of extremity and trunk desmoid tumors. Arch Orthop Trauma Surg. 2009;129(7):929–33.

    PubMed  Google Scholar 

  13. Yao X, Corbett T, Gupta AA, Kandel RA, Verma S, Werier J, et al. A systematic review of active treatment options in patients with desmoid tumours. Curr Oncol. 2014;21(4):e613-629.

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Wang J, Huang Y, Sun Y, Ge Y, Zhang M. Value of imaging findings in predicting post-operative recurrence of desmoid-type fibromatosis. Oncol Lett. 2020;19(1):869–75.

    CAS  PubMed  Google Scholar 

  15. Gronchi A, Colombo C, Le Péchoux C, Dei Tos AP, Le Cesne A, Marrari A, et al. Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm–a position paper from the Italian and the French Sarcoma Group. Ann Oncol. 2014;25(3):578–83.

    CAS  PubMed  Google Scholar 

  16. Kasper B, Baumgarten C, Bonvalot S, Haas R, Haller F, Hohenberger P, et al. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients' and professionals' expertise - a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. European journal of cancer (Oxford, England : 1990). 2015; 51(2):127–136.

  17. Pignatti G, Barbanti-Bròdano G, Ferrari D, Gherlinzoni F, Bertoni F, Bacchini P, et al. Extraabdominal desmoid tumor. A study of 83 cases. Clin Orthop Relat Res. 2000(375):207–213.

  18. von Mehren M, Randall RL, Benjamin RS, Boles S, Bui MM, Conrad EU, 3rd, et al. Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2016; 14(6):758–786.

  19. Bonvalot S. Sporadic abdominal wall desmoid: is it time to change our first-line approach? Ann Surg Oncol. 2014;21(7):2117–8.

    PubMed  Google Scholar 

  20. Kloeze J, van Veen M. Spontaneous regression of a sporadic intra-abdominal located desmoid-type fibromatosis. J Surg Case Rep. 2019; 2019(2):rjz037.

  21. Colombo C, Miceli R, Le Péchoux C, Palassini E, Honoré C, Stacchiotti S, et al. Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients. European journal of cancer (Oxford, England : 1990). 2015; 51(2):186–192.

  22. Park JS, Nakache YP, Katz J, Boutin RD, Steffner RJ, Monjazeb AM, et al. Conservative management of desmoid tumors is safe and effective. J Surg Res. 2016;205(1):115–20.

    PubMed  Google Scholar 

  23. van Broekhoven DLM, Verschoor AJ, van Dalen T, Grünhagen DJ, den Bakker MA, Gelderblom H, et al. Outcome of nonsurgical management of extra-abdominal, trunk, and abdominal wall desmoid-type fibromatosis: a population-based study in the Netherlands. Sarcoma. 2018;2018:5982575.

    PubMed  PubMed Central  Google Scholar 

  24. Briand S, Barbier O, Biau D, Bertrand-Vasseur A, Larousserie F, Anract P, et al. Wait-and-see policy as a first-line management for extra-abdominal desmoid tumors. J Bone Joint Surg Am. 2014;96(8):631–8.

    PubMed  Google Scholar 

  25. Duhil de Bénazé G, Vigan M, Corradini N, Minard-Colin V, Marie-Cardine A, Verite C, 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(7):1294–1300.

  26. Murphey MD, Ruble CM, Tyszko SM, Zbojniewicz AM, Potter BK, Miettinen M. From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 2009;29(7):2143–73.

    PubMed  Google Scholar 

  27. Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol. 2006;186(1):247–54.

    PubMed  Google Scholar 

  28. Rhim JH, Kim JH, Moon KC, Park SW, Sohn CH, Choi SH, et al. Desmoid-type fibromatosis in the head and neck: CT and MR imaging characteristics. Neuroradiology. 2013;55(3):351–9.

    PubMed  Google Scholar 

  29. Robbin MR, Murphey MD, Temple HT, Kransdorf MJ, Choi JJ. Imaging of musculoskeletal fibromatosis. Radiographics. 2001;21(3):585–600.

    CAS  PubMed  Google Scholar 

  30. Kransdorf MJ, Jelinek JS, Moser RP, Jr., Utz JA, Hudson TM, Neal J, et al. Magnetic resonance appearance of fibromatosis. A report of 14 cases and review of the literature. Skeletal Radiol. 1990; 19(7):495–499.

  31. Shinagare AB, Ramaiya NH, Jagannathan JP, Krajewski KM, Giardino AA, Butrynski JE, et al. A to Z of desmoid tumors. AJR Am J Roentgenol. 2011;197(6):W1008-1014.

    PubMed  Google Scholar 

  32. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. European journal of cancer (Oxford, England : 1990). 2020; 127:96–107.

  33. Basu S, Nair N, Banavali S. Uptake characteristics of fluorodeoxyglucose (FDG) in deep fibromatosis and abdominal desmoids: potential clinical role of FDG-PET in the management. Br J Radiol. 2007;80(957):750–6.

    CAS  PubMed  Google Scholar 

  34. Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal primary fibromatosis: aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008;34(4):462–8.

    CAS  PubMed  Google Scholar 

  35. Dalén BP, Geijer M, Kvist H, Bergh PM, Gunterberg BU. Clinical and imaging observations of desmoid tumors left without treatment. Acta Orthop. 2006;77(6):932–7.

    PubMed  Google Scholar 

  36. Garcia-Ortega DY, Martín-Tellez KS, Cuellar-Hubbe M, Martínez-Said H, Álvarez-Cano A, Brener-Chaoul M, et al. Desmoid-type fibromatosis. Cancers (Basel). 2020; 12(7).

  37. Kim Y, Rosario MS, Cho HS, Han I. Factors associated with disease stabilization of desmoid-type fibromatosis. Clin Orthop Surg. 2020;12(1):113–9.

    PubMed  PubMed Central  Google Scholar 

  38. Tanaka K, Yoshikawa R, Yanagi H, Gega M, Fujiwara Y, Hashimoto-Tamaoki T, et al. Regression of sporadic intra-abdominal desmoid tumour following administration of non-steroidal anti-inflammatory drug. World J Surg Oncol. 2008;6:17.

    PubMed  PubMed Central  Google Scholar 

  39. Sueishi T, Arizono T, Nishida K, Hamada T, Inokuchi A. A case of spontaneous regression of recurrent desmoid tumor originating from the internal obturator muscle after delivery. World J Oncol. 2016;7(4):75–80.

    PubMed  PubMed Central  Google Scholar 

  40. Castellazzi G, Vanel D, Le Cesne A, Le Pechoux C, Caillet H, Perona F, et al. Can the MRI signal of aggressive fibromatosis be used to predict its behavior? Eur J Radiol. 2009;69(2):222–9.

    CAS  PubMed  Google Scholar 

  41. Cassidy MR, Lefkowitz RA, Long N, Qin LX, Kirane A, Sbaity E, et al. Association of MRI T2 signal intensity with desmoid tumor progression during active observation: a retrospective cohort study. Ann Surg. 2020;271(4):748–55.

    PubMed  Google Scholar 

  42. Vandevenne JE, De Schepper AM, De Beuckeleer L, Van Marck E, Aparisi F, Bloem JL, et al. New concepts in understanding evolution of desmoid tumors: MR imaging of 30 lesions. Eur Radiol. 1997;7(7):1013–9.

    CAS  PubMed  Google Scholar 

  43. Gondim Teixeira PA, Biouichi H, Abou Arab W, Rios M, Sirveaux F, Hossu G, et al. Evidence-based MR imaging follow-up strategy for desmoid-type fibromatosis. Eur Radiol. 2020;30(2):895–902.

    CAS  PubMed  Google Scholar 

  44. Mahnashi Y, Alotaibi AS, Aldakhail M, Alshahrani A. Desmoid tumor at the gastrointestinal anastomosis after a one-anastomosis gastric bypass (mini-gastric bypass): a case report. J Surg Case Rep. 2020; 2020(1):rjz411.

  45. Samejima J, Ito H, Yokose T, Nagashima T. Desmoid-type fibromatosis arising from thoracotomy incision. Clin Case Rep. 2020;8(2):389–90.

    PubMed  Google Scholar 

  46. Ohtake S, Namura K, Fujikawa A, Sawada T, Ohta J, Moriyama M, et al. A case of abdominal wall desmoid tumor after radical nephrectomy for renal cancer. Hinyokika Kiyo. 2015;61(9):353–7.

    PubMed  Google Scholar 

  47. Kim PS, Han DS, Han HS, Bang HY. Intra-abdominal fibromatosis after gastrectomy for gastric cancer. Ann Surg Treat Res. 2014;87(6):331–5.

    PubMed  PubMed Central  Google Scholar 

  48. Nałęcz A, Dębski K, Dobosz M, Krauze M. Desmoid tumor of the mesentery in a patient after restorative proctocolectomy as a result of familial adenomatous polyposis - case reports. Pol Przegl Chir. 2018;90(3):53–8.

    PubMed  Google Scholar 

  49. Mori T, Yamada T, Ohba Y, Yoshimoto K, Ikeda K, Shiraishi K, et al. A case of desmoid-type fibromatosis arising after thoracotomy for lung cancer with a review of the english and Japanese literature. Ann Thorac Cardiovasc Surg. 2014;20(Suppl):465–9.

    PubMed  Google Scholar 

  50. Gupta R, Andley M, Talwar N, Kumar A. Sporadic desmoid-type fibromatosis occurring at laparoscopic trocar site: an unusual entity. Indian J Surg. 2018;80(3):275–7.

    PubMed  Google Scholar 

  51. Navin P, Lehrke HD, Schmitz JJ, Truty MJ, Moynagh MR. Desmoid fibromatosis mimicking metastatic recurrence after pancreatectomy for pancreatic adenocarcinoma. Mayo Clin Proc Innov Qual Outcomes. 2018;2(4):392–7.

    PubMed  PubMed Central  Google Scholar 

  52. Jiang D, He D, Hou Y, Lu W, Shi Y, Hu Q, et al. Subsequent intra-abdominal fibromatosis mimicking recurrent gastrointestinal stromal tumor. Diagn Pathol. 2013;8:125.

    PubMed  PubMed Central  Google Scholar 

  53. Kim JH, Ryu MH, Park YS, Kim HJ, Park H, Kang YK. Intra-abdominal desmoid tumors mimicking gastrointestinal stromal tumors - 8 cases: a case report. World J Gastroenterol. 2019;25(16):2010–8.

    PubMed  PubMed Central  Google Scholar 

  54. Fleetwood VA, Zielsdorf S, Eswaran S, Jakate S, Chan EY. Intra-abdominal desmoid tumor after liver transplantation: a case report. World J Transplant. 2014;4(2):148–52.

    PubMed  PubMed Central  Google Scholar 

  55. Okamura A, Takahashi T, Saikawa Y, Mayanagi S, Fukuda K, Nakamura R, et al. Intra-abdominal desmoid tumor mimicking gastric cancer recurrence: a case report. World J Surg Oncol. 2014;12:146.

    PubMed  PubMed Central  Google Scholar 

  56. Nakanishi K, Shida D, Tsukamoto S, Ochiai H, Mazaki J, Taniguchi H, et al. Multiple rapidly growing desmoid tumors that were difficult to distinguish from recurrence of rectal cancer. World J Surg Oncol. 2017;15(1):180.

    PubMed  PubMed Central  Google Scholar 

  57. Ono H, Hori K, Tashima L, Tsuruta T, Nakatsuka SI, Ito K. A case of retroperitoneal desmoid-type fibromatosis that involved the unilateral ureter after gynaecologic surgery. Int J Surg Case Rep. 2018;47:30–3.

    PubMed  PubMed Central  Google Scholar 

  58. Wang P, Zhou H, Zhou Z, Liang J. Mesenteric fibromatosis misdiagnosed with lymph node metastasis after successful laparoscopic right hemicolectomy: a report of two cases with review of literature. Onco Targets Ther. 2018;11:4811–6.

    PubMed  PubMed Central  Google Scholar 

  59. Chu Y, Guo Q, Wu D. Mesenteric fibromatosis after resection for gastrointestinal stromal tumor of stomach: a case report. Medicine (Baltimore). 2017;96(48): e8792.

    PubMed  Google Scholar 

  60. Rosa F, Martinetti C, Piscopo F, Buccicardi D, Schettini D, Neumaier CE, et al. Multimodality imaging features of desmoid tumors: a head-to-toe spectrum. Insights Imaging. 2020;11(1):103.

    CAS  PubMed  PubMed Central  Google Scholar 

  61. Sabharwal S, Ahlawat S, Levin AS, Meyer CF, Brooks E, Ligon J, et al. Desmoid tumor mimics local recurrence of extremity sarcoma on MRI. J Surg Oncol. 2020;121(8):1259–65.

    PubMed  Google Scholar 

  62. Lee JH, Song KD, Cha DI, Hyun SH. New intra-abdominal mass after operation for colorectal cancer: desmoid tumor versus peritoneal seeding. Abdom Radiol (NY). 2018;43(11):2923–7.

    PubMed  Google Scholar 

  63. Nieuwenhuis MH, Casparie M, Mathus-Vliegen LM, Dekkers OM, Hogendoorn PC, Vasen HF. A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses. Int J Cancer. 2011;129(1):256–61.

    CAS  PubMed  Google Scholar 

  64. Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol. 1990; 14(4):335–341.

  65. Lotfi AM, Dozois RR, Gordon H, Hruska LS, Weiland LH, Carryer PW, et al. Mesenteric fibromatosis complicating familial adenomatous polyposis: predisposing factors and results of treatment. Int J Colorectal Dis. 1989;4(1):30–6.

    CAS  PubMed  Google Scholar 

  66. Libertini M, Mitra I, van der Graaf WTA, Miah AB, Judson I, Jones RL, et al. Aggressive fibromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response. Clin Sarcoma Res. 2018;8:13.

    CAS  PubMed  PubMed Central  Google Scholar 

  67. Gondim Teixeira PA, Chanson A, Verhaeghe JL, Lecocq S, Louis M, Hossu G, et al. Correlation between tumor growth and hormonal therapy with MR signal characteristics of desmoid-type fibromatosis: a preliminary study. Diagn Interv Imaging. 2019;100(1):47–55.

    CAS  PubMed  Google Scholar 

  68. Ingley KM, Burtenshaw SM, Theobalds NC, White LM, Blackstein ME, Gladdy RA, et al. Clinical benefit of methotrexate plus vinorelbine chemotherapy for desmoid fibromatosis (DF) and correlation of treatment response with MRI. Cancer Med. 2019;8(11):5047–57.

    CAS  PubMed  PubMed Central  Google Scholar 

  69. Zanchetta E, Ciniselli CM, Bardelli A, Colombo C, Stacchiotti S, Baldi GG, et al. Magnetic resonance imaging patterns of tumor response to chemotherapy in desmoid-type fibromatosis. Cancer Med. 2021;10(13):4356–65.

    CAS  PubMed  PubMed Central  Google Scholar 

  70. Zhu HC, Xu SX, Li XT, Guan Z, Li S, Sun YS. MRI T1 contrast-enhanced signal intensity is a prognostic indicator of imatinib therapy in Desmoid-type fibromatosis. Front Oncol. 2021;11: 615986.

    PubMed  PubMed Central  Google Scholar 

  71. Subhawong TK, Feister K, Sweet K, Alperin N, Kwon D, Rosenberg A, et al. MRI volumetrics and image texture analysis in assessing systemic treatment response in extra-abdominal desmoid fibromatosis. Radiol Imaging Cancer. 2021;3(4): e210016.

    PubMed  PubMed Central  Google Scholar 

  72. Kasper B, Dimitrakopoulou-Strauss A, Pilz LR, Strauss LG, Sachpekidis C, Hohenberger P. Positron emission tomography as a surrogate marker for evaluation of treatment response in patients with desmoid tumors under therapy with imatinib. Biomed Res Int. 2013;2013: 389672.

    PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

We thank Jane Charbonneau, DVM from Edanz (https://jp.edanz.com/ac), for editing a draft of this manuscript.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Miho Okuda.

Ethics declarations

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Okuda, M., Yoshida, K., Kobayashi, S. et al. Desmoid-type fibromatosis: imaging features and course. Skeletal Radiol 52, 1293–1303 (2023). https://doi.org/10.1007/s00256-023-04275-x

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00256-023-04275-x

Keywords

Navigation