An unusual cause of spontaneous recurrent pneumothoraces

Epithelioid sarcoma is a rare distinctive soft tissue neoplasm with a predilection for the distal parts of the extremities. While epithelioid sarcoma presenting as median nerve palsy or carpal tunnel syndrome has been described, epithelioid sarcoma presenting as cystic lung metastases has not been reported. This article reports a case of a man who presented with spontaneous recurrent pneumothoraces and was found to have bilateral cystic lung disease, which was ultimately diagnosed as epithelioid sarcoma with cystic lung metastases. He underwent left arm amputation, chemotherapy and radiation, but succumbed to his advanced disease.