Primary mediastinal clear cell sarcoma: a case report and review of the literature
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CASE REPORT
Open Access
Primary mediastinal clear cell sarcoma: a case report and review of the literature Long Jin1, Yuxia Sui2, Haili Zhu3, Zhizhong Chen1 and Shuguang Liu4*
Abstract Background: Clear cell sarcoma (CCS) is a rare malignant soft-tissue neoplasm that displays melanocytic markers and exhibits striking histopathological features. The tumour has a predilection for the lower extremities and rarely presents in the mediastinum. Case presentation: We present a case of primary mediastinal CCS in a 57-year-old man. Computer tomography (CT) revealed a 12 × 12 × 7.5 cm mass in the anterior mediastinum. Microscopically, the tumour mainly consisted of epithelioid cells with oval vesicular nuclei and eosinophilic cytoplasm. Immunohistochemically, the tumour was positive for human melanoma black 45 (HMB-45) and vimentin but negative for S-100 and Melan-A. Fluorescence in situ hybridisation (FISH) showed a translocation involving the EWSR1 gene region. Conclusion: This report will illustrate that the mediastinum is a potential site for primary CCS and FISH plays an important role in making a conclusive diagnosis. Keywords: Clear cell sarcoma, Mediastinum, Fluorescence in situ hybridisation, EWSR1
Background CCS is a rare aggressive sarcoma accounting for only 1% of soft-tissue sarcoma. It usually occurs in adolescents and young adults. The first description of a case of CCS is attributed to Enzinger in 1965, which called it a “malignant melanoma of soft parts” due to its melanocytic differentiation [1]. The tumour often arises in deep soft tissue of the lower extremities, especially the region of the foot and ankle, in association with fascia, tendons, or aponeuroses. It is rarely located in visceral sites [2–7]. Common tumours in adults that occur in the anterior mediastinum are thymomas, lymphomas, and germ cell tumours. Other soft-tissue tumours that arise in the anterior mediastinum can include leiomyosarcoma, malignant peripheral nerve sheath tumour, liposarcoma, synovial sarcoma, angiosarcoma, and extraskeletal osteosarcoma, among others. A primary CCS in the anterior mediastinum is extremely rare. The present study reports an unusual case of CCS in the mediastinum, confirmed by FISH analysis and immunohistochemical study. * Correspondence: [email protected] 4 Department of Pathology, The Eighth Affiliated Hospital, Sun Yat-sen University, 3025 Shennan Middle Road, Shenzhen 518033, Guangdong Province, People’s Republic of China Full list of author information is available at the end of the article
Case presentation A 57-year-old man was admitted to our hospital with complaints of a 1-month history of chest distending pain. He had no history of cutaneous malignancy. On admission, a chest CT revealed a giant lobulated mass with uneven density approximately 12 × 12 × 7.5 cm in size occupying the anterior mediastinum (Fig. 1a and b). There were no obvious calcification and fat density shadows. The mass showed a slight heterogeneous enhancement on a contrast-enhanced CT scan. The serum levels of tumour markers
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