Dermatofibrosarcomul protuberans fibrosarcomatos retroauricular stâng. Prezentare de caz

Purpose. Dermatofibrosarcoma protuberans is a malignant skin tumor that occurs in the dermis and is characterized by slow infiltrative growth, low metastatic potential, but with a high tendency to local recurrence after the surgical treatment. Methods. We present the case of a 36-year-old patient who was admitted to the ENT clinic of “Dr. Carol Davila” Universitary Central Military Hospital in July 2019 for the appearance of a left retroauricular tumor formation, operated in May 2019 in another ENT department. The histopathological test estabilished the diagnosis of dermatofibrosarcoma protuberans. The patient was hospitalized for further surgical treatment. Results. The ENT clinical examination revealed a well-defined retroauricular tumor formation of about 3 cm, of fibrous consistency, painless spontaneously and on palpation, with vascularization, adherent to the superficial and deep planes, which flattened the tip of the mastoid. The contrast-enhanced cervical MRI revealed a remaining tumor measuring 15/17 mm in the axial plane and 25 mm in the caudal plane, with homogeneous contrast sockets, located retroauricularly on the left side, attached to the anterior wall of the mastoid, the lesion coming in contact with the parotid gland to which there was no demarcation limit. It was decided and performed the excision of the left retroauricular tumor, and the immunohistochemical examination established the diagnosis of fibrosarcomatous dermatofibrosarcoma. The patient underwent a reoperation at 2 months postoperatively consisting of radical excision of the left parotid gland, due to tumor infiltration revealed by HP examination. There were no signs of recurrence at one year postoperatively. Conclusions. Fibrosarcomatous dermatofibrosarcoma is the most aggressive subtype and has a high metastatic potential. In front of a young patient, the clinician must always maintain a high degree of suspicion of a possible malignant cause, and the defi­nite diagnosis must be supported by the histopathological exa­mi­na­tion. The multidisciplinary team is essential in fi­na­li­zing a correct and complete treatment for the patient.