Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients

• Published in: European journal of surgical oncology 2008-04, Vol.34 (4), p.462-468
• Main Authors: Bonvalot, S, Eldweny, H, Haddad, V, Rimareix, F, Missenard, G, Oberlin, O, Vanel, D, Terrier, P, Blay, J.Y, Le Cesne, A, Le Péchoux, C
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aggressive fibromatosis; Antineoplastic Agents - therapeutic use; Child; Child, Preschool; Combined Modality Therapy; Desmoid tumour; Female; Fibromatosis, Aggressive - pathology; Fibromatosis, Aggressive - surgery; Fibromatosis, Aggressive - therapy; Hematology, Oncology and Palliative Medicine; Humans; Infant; Male; Middle Aged; Radiotherapy, Adjuvant; Soft Tissue Neoplasms - pathology; Soft Tissue Neoplasms - surgery; Soft Tissue Neoplasms - therapy; Soft tissue tumour; Surgery; Survival Analysis
• ISSN: 0748-7983; 1532-2157
• DOI: 10.1016/j.ejso.2007.06.006

Abstract Objective To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis. Patients and methods 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. Results 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). Conclusions 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.