[Pulmonary metastasis of a dermatofibrosarcoma].

Progressive and recurrent dermatofibrosarcoma, described by Darier and Ferrand in 1924, is a fibrous skin tumour with essentially local malignancy. The authors report a case with pulmonary metastasis, a rare event as only 13 cases of visceral metastases have been reported in the literature. The clinical course of this case was favourable (follow-up of 5 years), in contrast with the usually pejorative nature of metastatic disease (death after several months to one year following the discovery of the first metastasis).