Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group.

@article{Gronchi2014SporadicDF,
  title={Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group.},
  author={Alessandro Gronchi and Chiara Colombo and C{\'e}cile Le P{\'e}choux and Angelo Paolo Dei Tos and Axel Le Cesne and Andrea Marrari and Nicolas Penel and Giovanni Grignani and J. Y. Blay and Paolo Giovanni Casali and Eberhard Stoeckle and Franco Gherlinzoni and Pierre M{\'e}eus and Chiara Mussi and François Gouin and Florence Duffaud and Michele Fiore and Sylvie Bonvalot},
  journal={Annals of oncology : official journal of the European Society for Medical Oncology},
  year={2014},
  volume={25 3},
  pages={
          578-83
        }
}
  • A. Gronchi, C. Colombo, S. Bonvalot
  • Published 1 March 2014
  • Medicine
  • Annals of oncology : official journal of the European Society for Medical Oncology
Desmoid-type fibromatosis (DF) is a rare locally aggressive monoclonal proliferation of myofibroblasts lacking metastatic capacity. It may be observed in nearly every part of the body. Considering the variable clinical presentations, anatomic locations, and biologic behaviors, an individualized treatment approach is required. The pathogenesis of DF is not completely understood even if a high prevalence (∼85%) of CTNNB1 mutations discovered in sporadic DF underlies the importance of the Wnt… 

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References

SHOWING 1-10 OF 55 REFERENCES

The treatment of desmoid tumors: a stepwise clinical approach.

TLDR
An algorithm is proposed that commences with more conservative approaches before treatments that have associated long-term morbidity, the more aggressive therapies being reserved only for those who really need it.

Desmoid-type fibromatosis: what works best?

Desmoid tumor: a disease opportune for molecular insights.

TLDR
In an era of molecularly targeted therapeutics there is a real need to better grasp the molecular mechanisms behind desmoid tumorigenesis and progression, to result in the development of patient and tumor tailored therapies and assist in the control and eradication of this disease.

Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up.

  • N. PenelA. Le Cesne J. Blay
  • Medicine
    Annals of oncology : official journal of the European Society for Medical Oncology
  • 2011
TLDR
Imatinib is active in the treatment of recurrent and progressive AF/DT, providing objective response and long-term stable disease in a large proportion of patients.

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

TLDR
A conservative policy could be a safe approach to primary and recurrent DF, which could avoid unnecessary morbidity from surgery and/or radiation therapy, and a multidisciplinary, stepwise approach should be prospectively tested in DF.

Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation.

  • S. SalasA. Dufresne J. Coindre
  • Medicine
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • 2011
TLDR
This study clearly demonstrates that there are different prognostic subgroups of desmoid tumors that could benefit from different therapeutic strategies, including a wait-and-see policy.

Efficacy of Imatinib in Aggressive Fibromatosis: Results of a Phase II Multicenter Sarcoma Alliance for Research through Collaboration (SARC) Trial

TLDR
Imatinib may have a role in the management of unresectable or difficult to resect desmoid tumors as well as evaluations undertaken to examine the mechanism of imatinib.

Desmoid tumors: need for an individualized approach

TLDR
Treatment of desmoid tumor is individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life, and systemic treatment may be indicated in patients that have anatomic barriers to effective surgery or radiotherapy.

Activity of toremifene in sporadic desmoid-type fibromatosis.

  • M. FioreC. Colombo A. Gronchi
  • Medicine
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • 2011
TLDR
Toremifene seems active and well tolerated in DF andSymptomatic relief is often obtained and previous progression on tamoxifen should not be considered a contraindication to T.
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