ABSTRACT
A 49-year-old man presented with a rare dermatofibrosarcoma protuberans (DFSP) of the scalp associated with local recurrence and distant metastasis to the lung and abdomen. An elastic-hard small mass on the right occipital scalp was initially treated by simple resection in another clinic. Ten years later, recurrent tumor was associated with infiltration to the calvarium, and resection was performed again also in another clinic. Approximately 1.5 years later, the patient was transferred to our clinic because of recurrence with intracranial involvement. Repeated relapses and metastasis to the lung were recognized despite surgery, chemotherapy, and local radiation. Eventually, the patient died of distant metastasis to the abdomen 17 years after the initial diagnosis. Scalp DFSP is very uncommon but is an aggressive scalp tumor, so initial wide local resection and local radiation therapy after surgery are important to prevent local recurrence and distant metastasis.
