Dilemma in diagnosis and management of rare primary pleural Ewing’s sarcoma with synchronous limited metastatic disease

We present a case of a 34-year-old woman who presented with complaints of fever, cough and dyspnoea of 2 months’ duration. On evaluation, she was diagnosed with a rare entity primary pleural Ewing’s sarcoma with synchronous metastases to mediastinal, supraclavicular nodes and single vertebra. Due to the rarity of this entity and lack of treatment guidelines on extraosseous Ewing’s sarcoma, the patient was managed with a combination of multiagent chemotherapy, surgery and radiotherapy as per standard guidelines for skeletal Ewing’s sarcoma. We present this case to discuss differential diagnoses and management dilemmas encountered on the use of local modalities such as surgery and radiotherapy for control of primary and metastatic sites.