Background: Desmoid tumor lately referred as aggressive fibromatosis (AF) is rare clinically heterogeneous and unpredictable disease with prevalence < 0.03% of all malignancies. The current standard treatment is: radical surgical treatment with tumour excision accompanied by radiotherapy and/or systemic approaches. The potential morbidity and the high local recurrence rates leaded investigators to assess the role of pharmacological approaches. The purpose of this review was to overview effectiveness of treatment options for AF and the role of imatinib mesylate (Glivec™; Novartis), in AF that has been published over the past decade.
Material and Methods: Published clinical trials, studies, case reports and case series literature review, was carried out using the electronic databases PubMed/Medline from 2002 to 2012.
Results: Reviewed literature included case reports, case series, retrospective studies and several small phase II trials. According to the review NSAIDs treatment performed positive response range of 37%- 57%, COX 2 treatment (Meloxicam) up to 90% response, treatment with hormonal agents response ratio between 40%-50%, positive responses for chemotherapy against AF of almost 50% and response rate for Imatinib ~10%. However several questions need to be answered: which is the most suitable treatment at certain AF condition, what is the optimal dose and duration of treatment since the lack of sufficient patient numbers and randomized trials compromises the validity of the reported results.
Conclusions: Reviewed literature presents evidence that citotoxic and non-citotoxic systemic therapies other that surgery are effective against AF. Main issue that has to be processed is stronger validation of results by further investigation with precisely designed prospective studies with larger patient numbers and, with main end points that include tumor respones rate and survival as well as quality of life.