This study describes a case of aggressive fibromatosis of the mediasti
nal cavity, symptomatic of considerable endothoracic growth and tissue
displacement. The case report illustrates the diffculties involved wi
th the rare semi-malignant disease in terms of both diagnostics and th
erapy. Since in this advaced stage surgery was only possible as R2 res
ektion, 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 g
iven the R2 situation, surgery was followed by renewed radiation thera
py. Based on an overview of the literature available, discussion focus
es on various etiologic theories at molecular and biological level and
histologic and genetic levels. A number of thera py approaches are pr
esented 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 num
bers 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.