Clear cell sarcoma of the mediastinum

• Published in: Annals of diagnostic pathology 2009-06, Vol.13 (3), p.197-200
• Main Authors: Tirabosco, Roberto, MD, Lang-Lazdunski, Loic, MD, PhD, FRCS, Diss, Timothy C., PhD, Amary, Maria Fernanda C., MD, PhD, Rodriguez-Justo, Manuel, MD, Landau, David, FRCP, Lorenzi, Will, MD, Flanagan, Adrienne M., MD, PhD, FRCPath
• Format: Article
• Language: English
• Subjects: Adenocarcinoma - pathology; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biomarkers, Tumor - analysis; Bone Neoplasms - drug therapy; Bone Neoplasms - secondary; Clear cell sarcoma; Diagnosis, Differential; EWS; Female; Gastrointestinal Stromal Tumors - pathology; Gene Rearrangement; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Lymphatic Metastasis - pathology; Mediastinal Neoplasms - genetics; Mediastinal Neoplasms - pathology; Mediastinal Neoplasms - therapy; Mediastinum; Middle Aged; Pathology; Reverse Transcriptase Polymerase Chain Reaction; RNA-Binding Protein EWS - genetics; Sarcoma, Clear Cell - genetics; Sarcoma, Clear Cell - secondary; Sarcoma, Clear Cell - therapy
• ISSN: 1092-9134; 1532-8198
• DOI: 10.1016/j.anndiagpath.2008.02.014

Abstract A 59-year-old woman presented with a large mediastinal mass. At thoracotomy, the mass was found tightly adherent to the esophageal wall and right lower lobe of the lung. Histological examination showed a solid tumor composed of closely packed nests of cells with clear and eosinophilic cytoplasm, which were strongly and diffusely positive for S100 protein but negative for HMB45 and Melan-A. The diagnosis of clear cell sarcoma was supported by demonstrating the presence of an EWS gene rearrangement by fluorescence in situ hybridization. There was no evidence that this lesion represented metastatic disease. To the best of our knowledge, primary mediastinal clear cell sarcoma has not been previously reported in the literature. We present the case and discuss the differential diagnosis.