PMC4659703_01
Initial presentation with massive swelling of right hand with involvement of hemithorax.
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As time progressed, so did the swelling with extension from her hand to shoulder with eventual involvement of her right chest wall and hemithorax (Figure 1).
Axial CT chest demonstrating swelling of right hemithorax.
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A computed tomography (CT) scan of the chest with contrast revealed soft tissue oedema without evidence of thrombus or obstruction in the venous system (Figure 2).
Pathology at 10x magnification showing a nodular, neoplastic proliferation composed of small ovoid to spindled cells demonstrating relatively bland, monomorphic nuclei and located within a variable fibrous to myxoid stroma.
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Immunohistochemically, the tumour cells involved were positive for epithelial membrane antigen and progesterone receptor but negative for Ewing sarcoma break point 1 gene translocation, smooth muscle antigen, cytokeratin AE1/AE3, S100, calretinin, microphthalmia-associated transcription factor (MITF), and claudin 1 (Figure 3).
Improvement in swelling after 1 cycle of chemotherapy.
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After one cycle of chemotherapy, she had a drastic improvement in her symptoms and physical appearance (Figure 4).