Strahlentherapie und Onkologie

, Volume 193, Issue 4, pp 269–275 | Cite as

Radiotherapy in desmoid tumors

Treatment response, local control, and analysis of local failures
  • Kirsi SanttiEmail author
  • Annette Beule
  • Laura Tuomikoski
  • Mikko Rönty
  • Anna-Stina Jääskeläinen
  • Kauko Saarilahti
  • Hanna Ihalainen
  • Maija Tarkkanen
  • Carl Blomqvist
Original Article



Desmoid tumors (aggressive fibromatosis) are rare soft tissue tumors which frequently recur after surgery. Desmoid tumors arise from musculoaponeurotic tissue in the extremities, head and neck, abdominal wall, or intra-abdominally. Our aim was to examine the outcome of radiotherapy of desmoid tumors in a single institution series.

Patients and methods

We evaluated 41 patients with desmoid tumors treated with 49 radiotherapies between 1987 and 2012. Radiologic images for response evaluation were reassessed and responses to treatment registered according to RECIST criteria 1.1. For patients with local failures radiation dose distribution was determined in each local failure volume using image co-registration. Recurrences were classified as in-target, marginal, or out-of-target. Prognostic factors for radiotherapy treatment failure were evaluated.


Radiotherapy doses varied from 20–63 Gy (median 50 Gy) with a median fraction size of 2 Gy. The objective response rate to definitive radiotherapy was 55% (12/22 patients). Median time to response was 14 months. A statistically significant dose-response relation for definitive and postoperative radiotherapy was observed both in univariate (p-value 0.002) and in multivariate analysis (p-value 0.02) adjusted for potential confounding factors. Surgery before radiotherapy or surgical margin had no significant effect on time to progression. Nine of 11 (82%) local failures were classified as marginal and two of 11 (18%) in-target. None of the recurrences occurred totally out-of-target.


Radiotherapy is a valuable option for treating desmoid tumors. Radiotherapy dose appears to be significantly associated to local control.


Aggressive fibromatosis Radiotherapy Local recurrence Dose-response relationship, radiation 

Radiotherapie bei Desmoid-Tumoren

Therapieansprechen, lokale Kontrolle und Lokalrezidivauswertung



Desmoide (aggressive Fibromatosen) sind seltene Weichteiltumore der muskulären Membranen von Kopf, Hals, Extremitäten und Bauchwand. Ziel war es, die Wirksamkeit der Strahlentherapie bei aggressiver Fibromatose an einer einzelnen Klinik zu untersuchen.

Patienten und Methode

Ausgewertet wurden 41 Patienten mit aggressiver Fibromatose, die zwischen 1987 und 2012 mit 49 Strahlentherapien behandelt wurden. Das Ansprechen wurde anhand der radiologischen Aufnahmen neu beurteilt und der Behandlungserfolg gemäß RECIST-1.1-Kriterien registriert. Für Patienten mit Lokalrezidiv wurde die Dosisverteilung der Radiotherapie durch Fusion von diagnostischen CT- und MRT-Bildern mit den Planungs-CT-Aufnahmen für jedes Lokalrezidiv bestimmt. Die Rezidive wurden je nach Lage eingeteilt in: innerhalb, am Rande und außerhalb des ehemaligen Bestrahlungsvolumens gelegen. Prognostische Faktoren für ein Therapieversagen wurden ausgewertet.


Die Gesamtdosen der Strahlentherapie lagen zwischen 20 und 63 Gy (Median 50 Gy) mit einer medianen Fraktionierung von 2 Gy. Die objektive Ansprechrate war 55 % (12/22 Patienten). Die mediane Zeit bis zum Therapieerfolg betrug 14 Monate. Eine statistisch signifikante Dosis-Wirkungs-Beziehung für die definitive und die postoperative Strahlentherapie wurde sowohl in der univariaten (p-Wert 0,002) als auch in der für potentielle Störfaktoren korrigierenden multivariaten Analyse (p-Wert 0,02) beobachtet. Neun von 11 (82 %) Lokalrezidiven wurden als Feldrandrezidive und zwei von 11 (18 %) als innerhalb des Bestrahlungsfelds liegende Rezidive bewertet. Kein Lokalrezidiv trat komplett außerhalb des Bestrahlungsfelds auf.


Die Strahlentherapie ist eine wertvolle Therapiewahl zur Behandlung aggressiver Fibromatosen. Die Strahlentherapiedosis hat einen signifikanten Einfluss auf die lokale Kontrolle.


Aggressive Fibromatose Radiotherapie Lokalrezidiv Dosis-Wirkungs-Beziehung, Bestrahlung 



Finska Läkaresällskapet supported the corresponding author and the study was supported from the EVO funds. We thank Annette Beule, MD PhD and Claudia Lundgren, MD for writing and editing the German abstract.

Compliance with ethical guidelines

Conflict of interest

K. Santti, A. Beule, L. Tuomikoski, M. Rönty, A.-S. Jääskeläinen, K. Saarilahti, H. Ihalainen, M. Tarkkanen, and C. Blomqvist declare that they have no competing interests.

Ethical standards

This article does not contain any studies with human participants or animals performed by any of the authors.

Supplementary material

66_2016_1091_MOESM1_ESM.docx (36 kb)
Supplementary data


  1. 1.
    Groen EJ, Roos A, Muntinghe FL et al (2008) Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol 15:2439–2450CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Heiskanen I, Jarvinen HJ (1996) Occurrence of desmoid tumours in familial adenomatous polyposis and results of treatment. Int J Colorectal Dis 11:157–162CrossRefPubMedGoogle Scholar
  3. 3.
    Cheon SS, Nadesan P, Poon R et al (2004) Growth factors regulate beta-catenin-mediated TCF-dependent transcriptional activation in fibroblasts during the proliferative phase of wound healing. Exp Cell Res 293:267–274CrossRefPubMedGoogle Scholar
  4. 4.
    Clark SK, Neale KF, Landgrebe JC et al (1999) Desmoid tumours complicating familial adenomatous polyposis. Br J Surg 86:1185–1189CrossRefPubMedGoogle Scholar
  5. 5.
    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 16:2587–2593CrossRefPubMedGoogle Scholar
  6. 6.
    Bonvalot S, Ternes N, Fiore M et al (2013) Spontaneous regression of primary abdominal wall desmoid tumors: more common than previously thought. Ann Surg Oncol 20:4096–4102CrossRefPubMedGoogle Scholar
  7. 7.
    Briand S, Barbier O, Biau D et al (2014) Wait-and-see policy as a first-line management for extra-abdominal desmoid tumors. J Bone Joint Surg Am 96:631–638CrossRefPubMedGoogle Scholar
  8. 8.
    Colombo C, Miceli R, Le Pechoux C et al (2015) Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients. Eur J Cancer 51:186–192CrossRefPubMedGoogle Scholar
  9. 9.
    Group ESESNW (2014) Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 25(Suppl 3):iii102–iii112CrossRefGoogle Scholar
  10. 10.
    Kasper B, Baumgarten C, Bonvalot S et al (2015) 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. Eur J Cancer 51:127–136CrossRefPubMedGoogle Scholar
  11. 11.
    von Mehren M, Benjamin RS, Bui MM et al (2016) NCCN Clinical Practise Guidelines in Oncology (NCCN Guidelines) Soft Tissue Sarcoma Version 2.2016Google Scholar
  12. 12.
    Escobar C, Munker R, Thomas JO et al (2012) Update on desmoid tumors. Ann Oncol 23:562–569CrossRefPubMedGoogle Scholar
  13. 13.
    Lazar AJ, Tuvin D, Hajibashi S et al (2008) Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol 173:1518–1527CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Lewis JJ, Boland PJ, Leung DH et al (1999) The enigma of desmoid tumors. Ann Surg 229:866–872 (discussion 872–3)CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Guadagnolo BA, Zagars GK, Ballo MT (2008) Long-term outcomes for desmoid tumors treated with radiation therapy. Int J Radiat Oncol Biol Phys 71:441–447CrossRefPubMedGoogle Scholar
  16. 16.
    Chao KS, Ozyigit G, Tran BN et al (2003) Patterns of failure in patients receiving definitive and postoperative IMRT for head-and-neck cancer. Int J Radiat Oncol Biol Phys 55:312–321CrossRefPubMedGoogle Scholar
  17. 17.
    Sampo MM, Tuomikoski L, Tarkkanen M et al (2013) Marginal miss or radioresistance? The pattern of local recurrence after operation and 3D planned radiation treatment in soft tissue sarcoma of the extremities and the limb girdles; an analysis based on image fusion. Acta Oncol 53(4):557–562. doi: 10.3109/0284186x.2013.844355 CrossRefPubMedGoogle Scholar
  18. 18.
    Eisenhauer EA, Therasse P, Bogaerts J et al (2009) New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer 45:228–247CrossRefPubMedGoogle Scholar
  19. 19.
    Alektiar K, Berry S (2011) Quantitative dosimetric analysis of patterns of local relapse after adjuvant IMRT for primary extremity sarcoma. Int J Radiat Oncol Biol Phys 81:S119 (Abstract)CrossRefGoogle Scholar
  20. 20.
    Gronchi A, Colombo C, Le Pechoux C et al (2014) 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 25:578–583CrossRefPubMedGoogle Scholar
  21. 21.
    Nuyttens JJ, Rust PF, Thomas CR Jr. et al (2000) Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 88:1517–1523CrossRefPubMedGoogle Scholar
  22. 22.
    Zlotecki RA, Scarborough MT, Morris CG et al (2002) External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys 54:177–181CrossRefPubMedGoogle Scholar
  23. 23.
    Gluck I, Griffith KA, Biermann JS et al (2011) Role of radiotherapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys 80:787–792CrossRefPubMedGoogle Scholar
  24. 24.
    Murphey MD, Ruble CM, Tyszko SM et al (2009) From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics 29:2143–2173CrossRefPubMedGoogle Scholar
  25. 25.
    Walker EA, Petscavage JM, Brian PL et al (2012) Imaging features of superficial and deep fibromatoses in the adult population. Sarcoma 2012:215810CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Yao X, Corbett T, Gupta AA et al (2014) A systematic review of active treatment options in patients with desmoid tumours. Curr Oncol 21:e613–e629CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Goy BW, Lee SP, Eilber F et al (1997) The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors. Int J Radiat Oncol Biol Phys 39:659–665CrossRefPubMedGoogle Scholar
  28. 28.
    Spear MA, Jennings LC, Mankin HJ et al (1998) Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys 40:637–645CrossRefPubMedGoogle Scholar
  29. 29.
    Ballo MT, Zagars GK, Pollack A et al (1999) Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 17:158–167CrossRefPubMedGoogle Scholar
  30. 30.
    Rüdiger HA, Ngan SYK, Ng M et al (2010) Radiation therapy in the treatment of desmoid tumours reduces surgical indications. Eur J Surg Oncol 36:84–88CrossRefPubMedGoogle Scholar
  31. 31.
    Keus RB, Nout RA, Blay JY et al (2013) 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 24(10):2672–2676. doi: 10.1093/annonc/mdt254 CrossRefPubMedGoogle Scholar
  32. 32.
    Ihalainen HR, Koljonen V, Bohling TO et al (2015) The desmoid tumour: local control after surgical treatment. J Plast Surg Hand Surg 49:19–24CrossRefPubMedGoogle Scholar
  33. 33.
    Baumert BG, Spahr MO, Von Hochstetter A et al (2007) The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network. Radiat Oncol 2:12CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Ballo MT, Zagars GK, Pollack A (1998) Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys 42:1007–1014CrossRefPubMedGoogle Scholar
  35. 35.
    Schulz-Ertner D, Zierhut D, Mende U et al (2002) The role of radiation therapy in the management of desmoid tumors. Strahlenther Onkol 178:78–83CrossRefPubMedGoogle Scholar
  36. 36.
    Schmitt G, Mills EE, Levin V et al (1992) Radiotherapy of aggressive fibromatosis. Eur J Cancer 28A:832–835CrossRefPubMedGoogle Scholar
  37. 37.
    Reitamo JJ, Hayry P, Nykyri E et al (1982) The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. Am J Clin Pathol 77:665–673CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Kirsi Santti
    • 1
    Email author
  • Annette Beule
    • 1
  • Laura Tuomikoski
    • 1
  • Mikko Rönty
    • 2
  • Anna-Stina Jääskeläinen
    • 1
  • Kauko Saarilahti
    • 1
  • Hanna Ihalainen
    • 3
  • Maija Tarkkanen
    • 1
  • Carl Blomqvist
    • 1
  1. 1.Comprehensive Cancer CenterHelsinki University Hospital and University of HelsinkiHelsinkiFinland
  2. 2.Department of PathologyHUSLAB and University of HelsinkiHelsinkiFinland
  3. 3.Department of Plastic SurgeryHelsinki University Hospital and University of HelsinkiHelsinkiFinland

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