Gigantic Dermatofibrosarcoma Protuberans of the Scalp with Fibrosarcomatous Transformation

Abstract

Here we are reporting this rare and unfortunate presentation of Dermatofibrosarcoma protuberans (DFSP) of the scalp. The patient was a 30 years old male from a remote area, with history of 4 previous surgical interventions. He was transferred directly to our surgical ICU with septicemia; anuria; severe anemia; and respiratory failure. Patient had a massive fungating infected tumor on his scalp. CT scan shown a subcutaneous scalp tumor with maximum diameter of 45 cm and adherence to occipital bone but no intracranial invasion. The tumor kept bleeding whenever the blood pressure maintained to optimum level. Patient had to be taken to the O.R joined with Neurosurgeon in attempt of surgical control of bleeding by elevating the tumor from its bed. Unfortunately, patient collapsed intra-operatively and despite extensive attempts of resuscitation by Anesthesia team, patient could not be salvaged. The final pathology was DFSP with malignant sarcomata’s transformation. To the best of our knowledge this is the largest reported DFSP case in the Head and Neck region, in English literature.