Fibrosarcomatous Dermatofibrosarcoma Protuberans of the Head

This article highlights the case of a 37-year-old male who presented with a recurrent, exponentially enlarging head mass, emphasizing the diagnostic and therapeutic challenges associated with a very rare type of tumor, fibrosarcomatous dermatofibrosarcoma protuberans (DFSP) of the head. Our patient presented with a rapidly growing head mass, initially diagnosed as a spindle cell tumor, and was managed with surgical excision and skin flap grafting. Follow-up revealed relapse and interval development of hemiparesis and hemisensory loss. MRI revealed tumor recurrence, with compression of the right parietal lobe and superior sagittal sinus. Histopathology revealed stroma with fascicles of spindle cells homogenous to fibrillar cytoplasm, with oval vesicular nuclei. Immunohistochemical staining showed positivity for CD34 and SMA. Oral chemotherapy with imatinib 800 mg/day was started. Follow-up imaging showed a marked reduction in the size of the tumor and resolution of the compression of the underlying brain parenchyma with cystic degeneration and decreased contrast enhancement. Future plans include possible surgical tumor debulking and/or radiation therapy. Although extremely rare, awareness of this tumor, with a multi-disciplinary approach to the management of the case, is vital to maximize treatment outcomes.