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


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


Kamal EL BAKRAOUI 1, Mohamed TARCHOULI2, Ibrahim ELGHISSASSI 1, Rhizlane BELBARAKA3, Hassan ERRIHANI1.

1Department of Medical Oncology, National Institute of Oncology, CHU Rabat, Morocco.
2Departement of Surgery, First Medical and Surgical Center, Agadir, Morocco.
3Department of Medical Oncology, FMPM, CHU Marrakech, Morocco.


International-Journal-of-Case-Reports-2d code

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.


Conflict of interest: The author declares that he has no conflict of interest


Keywords: Darierand Ferrand Dermatofibrosarcoma, Imatinib, Pulmonary Metastasis, Recurrence, Translocation.

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How to cite this article:

Kamal EL BAKRAOUI, Mohamed TARCHOULI, Ibrahim ELGHISSASSI, Rhizlane BELBARAKA, Hassan ERRIHANI.Pulmonary metastasis of a Dermatofibrosarcoma of Darier and Ferrand treated by IMATINIB: a case report. International Journal of Case Reports, 2019 4:76. DOI: 10.28933/ijcr-2019-04-2705


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