Dermatofibrosarcoma protuberans (DFSPs) is a low-grade mesenchymal tumour of dermal origin. Although lung metastasis is common, primary breast involvement is a very rare occurrence. The head and neck region, trunk and extremities are the common primary sites even though any site of the body may be potentially involved. Complete surgical resection with negative margins is the standard of care for nonmetastatic cases of DFSP. To the best of our knowledge, there have been no previous reports of metastatic DFSP of the breast to the lungs among male patients in sub-Saharan Africa. This case report presents data on a 32-year-old male with metastatic DFSP of the left breast to the lungs who was successfully treated with 800 mg daily imatinib mesylate therapy and achieved a complete radiological and clinical response after 1 year of palliative therapy.
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