Primary Mediastinal Embryonal Rhabdomyosarcoma in Adult: Literature Review and a Case Report

نویسندگان

  • Daniel Motola-Kuba
  • Ricardo Fernandez-Ferreira
چکیده

Rhabdomyosarcoma is extremely rare in adults, but is the most common extra cranial solid tumor in children. Rhabdomyosarcoma in adults is a heterogeneous disease, with different form of presentation, histology, and prognosis and with difficulty in its treatment due to the rarity of the disease. Here we briefly review the literature on adult rhabdomyosarcoma and present the case of a 39-year-old man clinical presentation of dyspnea, chest pain and weight loss, with initial approach with imaging studies and a mediastinal biopsy in which malignant neoplasia poorly differentiated with mesenchymal and epithelial immunophenotype is reported. He started chemotherapy with Paclitaxel/Carboplatin in a three-week regimen. After 2 cycles of chemotherapy a re-evaluation computed axial tomography showed progressive disease and the biopsy reported Embrionary Rhabdomyosarcoma with immunochemistry positive for myo D-1 and myogenine. Metastasis was confirmed at the diaphragm, retroperitoneal and central nervous system levels. 2nd line chemotherapy regimen with Vincristine, Doxorubicin and cyclophosphamide were initiated.

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تاریخ انتشار 2018