Aggressive fibromatosis - Desmoid tumor. Case report and overview of the literature

This study describes a case of aggressive fibromatosis of the mediastinal cavity, symptomatic of considerable endothoracic growth and tissue displacement. The case report illustrates the diffculties involved with the rare semi-malignant disease in terms of both diagnostics and therapy. Since in this advaced stage surgery was only possible as R2 resektion, a course of hormone treatment was carried out and followed up by consolidating radiation therapy. After more than three years during follow-up, the symtoms recurred locally and were re-operated. Again given the R2 situation, surgery was followed by renewed radiation therapy. Based on an overview of the literature available, discussion focuses on various etiologic theories at molecular and biological level and histologic and genetic levels. A number of thera py approaches are presented together with the experience gained from them. A total of 105 cases from 18 papers worldwide were collected for comparison purposes. In view of the extremely low incidence (0.0002-0.0004%) and the lack of uniformity of localization, there are very few papers on larger numbers of cases, so that congruency in assessment is rendered difficult. The summary of the studie reported reflects the variety of approaches taken in treatment and gives an overview of the results achieved.