Pulmonary Pathology

Clear Cell Sarcoma Presenting as Pleural Disease

Richard Attanoos
Cardiff, U.K


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Clinical History
A 37-year-old female, previously healthy, presented with a 2-month history of increasing dyspnea. CXR revealed a unilateral pleural effusion, and CT scan showed an effusion, chest wall/pleural mass, and pulmonary infiltrates. At thoracoscopy, the pleura appeared diffusely nodular. She underwent pleural biopsy.


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Figure 1
Sheets of small to intermediate hyperchromatic cells, undifferentiated.

Figure 2
Higher power reveals tumour cells have variable morphology. Some cells show vacuolated clear cytoplasm, other have pseudoinclusions of nuclei. In some, there is a plasmacytoid/ haematolymphoid appearance.

Figure 3
Focal positive AE1/AE3 (Cytokeratin).

Figure 4
Focal positive epithelial membrane antigen.

Figure 5
Focal positive CD138.

Figure 6
Weak focal expression of S100.

Figure 7
Focal positive HMB - 45.