IntroductionLes fibromatoses desmoïdes extra-abdominales (FDEA) sont des tumeurs bénignes rares, mais à un haut risque de récidive locale,
ce qui les distingue des autres tumeurs bénignes. Le but de ce travail était d’évaluer le taux de récidive locale, après traitement
chirurgical, afin de mieux définir les indications opératoires et les facteurs pronostiques de récidive.
MéthodeNous avons réalisé une étude rétrospective de 36 cas de tumeurs opérées dans notre établissement entre 1989 et 2008. Vingt-quatre
tumeurs ont récidivé. Les facteurs de risques évalués étaient l’âge, le sexe, la localisation de la tumeur, l’imagerie et
les marges de résection.
RésultatsCette tumeur touche surtout les femmes de la troisième ou quatrième décennie. Le principal facteur de récidive semble être
la qualité des marges de résection. L’IRM semble être utile pour prédire le potentiel de récidive de ces tumeurs après chirurgie
avec un facteur péjoratif en cas de lésions hypo-intenses en séquence T1 et hyper-intenses en T2 prenant la gadolinium.
DiscussionLa place de la chirurgie et des traitements associés (radiothérapie, chimiothérapie, tamoxifène) reste à déterminer et les
indications thérapeutiques doivent être précisées en fonction du rapport bénéfice/risque pour le patient. Enfin, la réalisation
d’une simple surveillance doit être évaluée, car de nombreuses lésions récidivantes se sont stabilisées spontanément.
Desmoid fibromatoses are a rare benign tumour but with a high rate of local recidivism. The goal of this article was to identify
the prognostic factor and to evaluate the efficacy of the surgery. We report 36 patients who underwent surgery in our department
between 1989 and 2008. Twenty-four had recidivism. This tumour primarily affects women in the third or fourth decade. The
principal prognostic factor is the margin of resection. MRI seems to be useful to predict the potential of recidivism. The
role of the surgery remains to be determined, and therapeutic indication must to be discussed with regard to benefits and
risks. Also, the “wait and see” attitude must be evaluated as this tumour stabilizes spontaneously.
Mots clésTumeur–Fibromatose desmoïde–Chirurgie
[Show abstract][Hide abstract] ABSTRACT: Eight patients with desmoid tumors, symptomatic, and none a candidate for conservative surgery, were treated with weekly vinblastine, maximum dose 10 mg/week, and methotrexate, maximum dose 50 mg/week. Symptomatic relief was obtained in all patients. Using Eastern Cooperative Oncology Group (ECOG) criteria, two patients had a complete remission, one of which has lasted for 30 months, four patients have had partial remissions, one patient has had a mixed response, and one patient who has been treated for only 4 weeks, a minimal response. Toxicity has been minor and transient. Chemotherapy appears to be an acceptable alternative to radical surgery in selected patients with desmoid tumors.
Cancer 10/1989; 64(6):1192-4. DOI:10.1002/1097-0142(19890915)64:63.0.CO;2-J · 4.89 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis.
Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy.
Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p=0.0002).
A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins.
European journal of surgical oncology: the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology 05/2008; 34(4):462-8. DOI:10.1016/j.ejso.2007.06.006 · 3.01 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The natural history of desmoid tumors remains an enigma. Previous reports attempting to identify their biology have included recurrent and primary tumors as well as tumors from both intra- and extra-abdominal sites. The purpose of this study was to analyze patients with primary extremity and trunk desmoid tumors treated and followed at a single institution and to determine factors influencing disease free survival.
Between July 1982 and June 1997, 189 patients with extremity and superficial trunk desmoid tumors were treated and followed prospectively. Of these, 105 presented with primary disease and formed the basis of this study.
The median follow-up for the entire group of patients was 49 months; it was 46 months for patients who did not develop a local recurrence. During this time, 24 patients (23%) had a local recurrence. No patients died of disease. The 2-year and 5-year local recurrence free survival rates were 80% and 75%, respectively. None of the prognostic factors analyzed, including age, gender, depth of tumor, size of tumor, or tumor site, were significant for predicting local recurrence. Moreover, positive resection margins were not predictive of recurrence. The selective use of adjuvant radiation therapy did not influence the rate of local recurrence regardless of the margin status.
Attempts to achieve negative resection margins may result in unnecessary morbidity and may not prevent local recurrence. Operations that preserve function and structure should be the primary goal, because the presence of residual disease cannot be clearly shown to impact adversely on 5-year disease free or overall survival.
Cancer 12/1999; 86(10):2045-52. DOI:10.1002/(SICI)1097-0142(19991115)86:103.0.CO;2-F · 4.89 Impact Factor
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