Abstract

The treatment of desmoid tumours (DTs) is controversial. Anti-oestrogen therapy has frequently been used, but clear information of its efficacy is lacking. In this systematic review we have undertaken a comprehensive analysis to assess the effectiveness of anti-oestrogen therapy in terms of ability to induce partial or complete regression of DTs. A systematic review of articles published in English between January 1983 and December 2009 was carried out according to the RECIST criteria. A literature search was performed on electronic databases including: United States National Library of Medicine (MEDLINE-PubMed), Excerpta Medica (EMBASE), Cochrane Library and Google search engine. Two-hundred articles dealing with DTs were identified but only fourty-one were were selected as appropriate for the study. The chi-square test was used for statistical analysis. Data on 168 DTs treated with anti-oestrogen agents, alone or in combination with nonsteroidal anti-inflammatory drugs, were identified with an overall response rate of 51%. There was no difference in response according to the type of DTs or between different anti-oestrogen therapies. Combination with anti-inflammatory drugs did not improve the outcome. Toremifene was sometimes effective in cases resistant to tamoxifen. Response did not seem to be related to oestrogen receptor status. Despite potential inaccuracies in the methodology, the results of the review indicate that anti-oestrogen therapy produces some effect in about one half of patients with DTs. Its indication compared with other treatments is discussed.

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