[Metastatic dermatofibrosarcoma: an unusual cause of mediastinal mass].
نویسندگان
چکیده
Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the soft tissue that is locally aggressive. Metastases are extremely uncommon and there are few cases published in the literature.1–4 We report the exceptional case of a mediastinal mass as the unique presentation of metastatic disease of a DFSP diagnosed by blind transbronchial needle aspiration (TBNA) with a histology needle. The patient is a 50-year-old male, a smoker, who in 2004 had been diagnosed with DFSP that was 7 cm in diameter and located in the left scapulohumeral region. The initial treatment was surgical, but after the resection the deep edges were affected. The patient had several local relapses that were treated surgically in 2006 and 2009. In the last surgical biopsy, the edges were tumor-free and the patient received no adjuvant treatment. Recently, the patient came for a consultation due to fever, arthralgia and unproductive cough. Radiography showed a paratracheal mass. CT confirmed the presence of a heterogeneous mediastinal mass with compression of the adjacent structures (Fig. 1). Bronchoscopy revealed edematous mucosa at the entrance of the main bronchus and the upper right lobe. Blind TBNA was performed with an MW-319 histology needle (Bard-Wang, Billerica, MA, USA) and the anatomopathologic result was DFSP metastasis, based on the morphology of the biopsies and supported by immunohistochemistry techniques (strong expression of vimentin and CD34) and by the genetic-molecular study (presence of translocation of chromosomes 17 and 22).
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ورودعنوان ژورنال:
- Archivos de bronconeumologia
دوره 47 11 شماره
صفحات -
تاریخ انتشار 2011