Annals of Surgical Oncology

, Volume 16, Issue 9, pp 2587–2593 | Cite as

Desmoid-Type Fibromatosis: A Front-Line Conservative Approach to Select Patients for Surgical Treatment

  • Marco Fiore
  • Françoise Rimareix
  • Luigi Mariani
  • Julien Domont
  • Paola Collini
  • Cecile Le Péchoux
  • Paolo G. Casali
  • Axel Le Cesne
  • Alessandro GronchiEmail author
  • Sylvie BonvalotEmail author
Bone and Soft Tissue Sarcomas



Surgery is still the standard treatment for desmoid-type fibromatosis (DF). Recently, the Institut Gustave Roussy (IGR), Villejuif, France, reported a series of patients treated with a front-line conservative approach (no surgery and no radiotherapy). The disease remained stable in more than half of patients. This study was designed to evaluate this approach on the natural history of the disease in a larger series of patients.


A total of 142 patients presenting to the IGR or Istituto Nazionale Tumori (INT), Milan, Italy, were initially treated using a front-line deliberately conservative policy. Their progression-free survival (PFS) was observed and a multivariate analysis was performed for major clinical variables.


Seventy-four patients presented with primary tumor, 68 with recurrence. Eighty-three patients received a “wait & see” policy (W&S), whereas 59 were initially offered medical therapy (MT), mainly hormonal therapy and chemotherapy. A family history of sporadic colorectal cancer was present in 8% of patients. The 5-year PFS was 49.9% for the W&S group and 58.6% for the medically treated patients (P = 0.3196). Similar results emerged for primary and recurrent DF. Multivariate analysis identified no clinical variables as independent predictors of PFS. In the event of progression, all patients were subsequently managed safely.


A conservative policy could be a safe approach to primary and recurrent DF, which could avoid unnecessary morbidity from surgery and/or radiation therapy. Half of patients had medium-term stable disease after W&S or MT. A multidisciplinary, stepwise approach should be prospectively tested in DF.


Medical Treatment Imatinib Mesylate Thoracic Wall Isolate Limb Perfusion Median Tumor Size 
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Copyright information

© Society of Surgical Oncology 2009

Authors and Affiliations

  • Marco Fiore
    • 1
  • Françoise Rimareix
    • 2
  • Luigi Mariani
    • 3
  • Julien Domont
    • 4
  • Paola Collini
    • 5
  • Cecile Le Péchoux
    • 6
  • Paolo G. Casali
    • 7
  • Axel Le Cesne
    • 4
  • Alessandro Gronchi
    • 1
    Email author
  • Sylvie Bonvalot
    • 2
    Email author
  1. 1.Department of SurgeryIstituto Nazionale TumoriMilanItaly
  2. 2.Department of SurgeryInstitut Gustave RoussyVillejuifFrance
  3. 3.Department of BiostatisticsIstituto Nazionale TumoriMilanItaly
  4. 4.Department of Medical OncologyInstitut Gustave RoussyVillejuifFrance
  5. 5.Department of PathologyIstituto Nazionale TumoriMilanItaly
  6. 6.Department of RadiotherapyInstitut Gustave RoussyVillejuifFrance
  7. 7.Department of Cancer MedicineIstituto Nazionale TumoriMilanItaly

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