METASTATIC PULMONARY DARIER AND FERRAND'S DERMATOFIBROSARCOMA: A CASE REPORT AND LITERATURE REVIEW
Meryem El Aamraoui*, Najwa Chebli, Dauphin Ntama, Salma Najem, Sihame Lkhoyaali, Sarah Naciri, Hanane Inrahoun, Saber Boutayeb, Ibrahim El Ghissassi, Hind Mrabti and Hassan Errihani
ABSTRACT
Dermatofibrosarcome de Darier et Ferrand (DFS) is a skin tumor with intermediate malignancy. Although rare, it represents about 0.1% of malignant skin tumors. The authors report the case of a 48-year-old patient who was admitted to the emergency department due to diffuse chest pain persisting for 3 months, without any other associated symptoms. The patient's medical history includes the excision of nodules located at the root of the left thigh, but there are no available records regarding this prior procedure. The diagnosis of dermatofibrosarcome de Darier et Ferrand was suspected during the standard histological examination and confirmed by the observation of intense and diffuse expression of the CD34 protein. The treatment involved surgery, in combination with chemotherapy. It is essential to perform a biopsy and histological examination to establish an accurate diagnosis. The prognosis of this tumor is primarily influenced by its potential for local malignancy and the high risk of recurrence. Transformation into a markedly malignant, metastasizing sarcomatous form is rare.
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