Malignant pleural mesothelioma (MPM) is a relatively rare but aggressive pleural tumor. Systemic sclerosis (SSc) is associated with the development of lung cancer and other malignancies. We describe a 58-year-old never-smoker female, previously diagnosed with limited cutaneous SSc, who presented with chest pain on the left side, dyspnea and circumferential nodular left pleural thickening. Fluorodeoxyglucose positron-emission tomography-computed tomography showed intense uptake in the thickened left pleura, mediastinal lymph nodes and left femur. Pleural fluid cytological examination along with nuclear and membranous positivity for Wilms tumor-1 antigen and epithelial membrane antigen on immunocytochemistry confirmed the diagnosis of MPM. To the best of our knowledge, this is the first report of MPM in a patient with SSc.
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