Pulmonary metastasis of a Dermatofibrosarcoma of Darier and Ferrand treated by IMATINIB: a case report

Abstract

Dermatofibrosarcoma (DFS) described by Darier and Ferrand represents less than 5% of soft tissue sarcomas and metastases only very rarely. We report the case of a 51-year-old patient followed for six years for a DFS of the right thigh root recurrent several times after non-optimal surgical resections. The occurrence of thoracic pain associated with a cough with dyspnea, motivated the realization of a thoracic computed tomography (CT) showing a right basal pulmonary mass with pulmonary nodules. CT biopsy of one of the pulmonary nodules confirmed the metastatic nature of these lesions. The search for translocation t (17,22) could not be performed. A treatment based on imatinib has been started. The evolution was marked by the disappearance of the respiratory symptomatology and a good radiological response. The advent of targeted therapy with imatinib transformed the prognosis for this disease, which was considered incurable at the metastatic stage.