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Review
. 2017 Jan 13;12(1):5.
doi: 10.1186/s13000-016-0594-z.

Primary mediastinal clear cell sarcoma: a case report and review of the literature

Long Jin  1 , Yuxia Sui  2 , Haili Zhu  3 , Zhizhong Chen  1 , Shuguang Liu  4
Affiliations
Review

Primary mediastinal clear cell sarcoma: a case report and review of the literature

Long Jin et al. Diagn Pathol. .

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; EWSR1; Fluorescence in situ hybridisation; Mediastinum.

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Figures

Fig. 1
Fig. 1
Chest computed tomography (CT). a, b The tumour mass was located in the anterior mediastinum with heterogeneous enhancement
Fig. 2
Fig. 2
a The tumour demonstrated expansive growth and infiltrated into the adjacent lung tissue (magnification × 40). b The tumour mainly consisted of epithelioid or polygonal cells arranged in sheets (magnification × 100). c Clusters of tumour cells were set in a fibrous stroma (magnification × 200). d Tumour cells have eosinophilic cytoplasm and oval vesicular nuclei with prominent eosinophilic nucleoli (magnification × 400). e A large necrosis was found (magnification × 100). f There were some melanin-producing tumour cells (magnification × 100)
Fig. 3
Fig. 3
a Immunohistochemical examination showed positive expression of vimentin (magnification × 400). b Immunohistochemical examination showed positive expression of HMB45 (magnification × 400). c Approximately 30% of the tumour cells were positive for the proliferation marker (Ki67, magnification × 200). d Dual color interphase fluorescence in situ hybridisation utilizing the EWSR1 break-apart probe. Split red and green signals within a single cell demonstrated the presence of a EWSR1 rearrangement (indicated by white arrows)
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References

    1. Enzinger FM. Clear cell sarcoma of tendons and aponeuroses: an analysis of 21 cases. Cancer. 1965;18:1163–74. doi: 10.1002/1097-0142(196509)18:9<1163::AID-CNCR2820180916>3.0.CO;2-0. - DOI - PubMed
    1. Comin CE, Novelli L, Tornaboni D, Messerini L. Clear cell sarcoma of the ileum: report of a case and review of literature. Virchows Arch. 2007;451:839–45. doi: 10.1007/s00428-007-0454-z. - DOI - PubMed
    1. Sabate JM, Fernandez A, Torrubia S, Villanueva A, Monill JM. Clear cell sarcoma of the abdominal wall with peritoneal sarcomatosis: CT features. Eur Radiol. 1999;9:1550–2. doi: 10.1007/s003300050882. - DOI - PubMed
    1. Katabuchi H, Honda R, Tajima T, Ohtake H, Kageshita T, Ono T, et al. Clear cell sarcoma arising in the retroperitoneum. Int J Gynecol Cancer. 2002;12:124–7. doi: 10.1046/j.1525-1438.2002.01052.x. - DOI - PubMed
    1. Bury T, Hermans G, Alexis-Agnant R, Chevalier P, Limet R, Bartsch P. Clear cell sarcoma: an extremely rare cause of pleural disease. Eur Respir J. 1997;10:2653–6. doi: 10.1183/09031936.97.10112653. - DOI - PubMed

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