Dermatofibrosarcoma protuberans: Review of the literature and report of four cases

The literature on dermatofibrosarcoma protuberans is reviewed. The origin of the tumor is still in doubt, some maintaining that it arises in mammary tissue, some stating that it is of neurogenic origin and others attributing an important role to trauma. Considerable controversy exists in regard to the histopathology of the lesion. It appears to us, as well as to many others, that the important feature is the non-specificity of the histological picture. As in the case of Kaposi's sarcoma, the clinical rather than the histopathological aspects are most important for diagnosis. The tumor is locally malignant, recurring after incomplete removal. Despite the fact that there have been reports of fatal metastases, in almost all these cases autopsy proof has been lacking. Four cases of DFSP are reported which showed the characteristic clinical features along with the non-specific histopathology previously described.