Peripheral primitive neuroectodermal tumor of the pleura in a 41-year-old female

Peripheral primitive neuroectodermal tumor (pPNET) is a rare, very aggressive neoplasm that belongs to a small round cell tumor, and most often arises from the chest wall. Here, we report a female case with proven pPNET who was treated in our institution. She presented with a history of left side chest pain, cough, and significant weight loss. Contrast enhanced CT imaging of the chest showed multiple left pleural-based enhancing masses with left diaphragmatic involvement. She underwent chemotherapy followed by tumor debulking through thoracotomy. However, she died of rapid growth from recurrent local tumors 3 months thereafter.