Epithelioid sarcoma with spontaneous pneumothorax and massive pleural effusion

Epithelioid sarcoma (ES) is an uncommon mesenchymal tumor that most frequently occurs in the dermal or subcutaneous area of the distal extremities of young adults, mainly in men. ES has a poor prognosis owing to its aggressiveness, as it frequently recurs locally and can undergo metastasis mainly to the lymph nodes, soft tissues, bones, lungs, and brain. Unilateral or bilateral spontaneous pneumothorax with cystic lung metastasis has been reported [1-5]. To our knowledge, only five patients have been reported in the literature, none of whom had massive pleural effusion. We reviewed the existing literature on pneumothorax in ES cases and present herein the first reported case of spontaneous pneumothorax and massive pleural effusion in both lungs in ES. KCI Citation Count: 5