Spontaneous Haemothorax as Initial Presentation of Pleural Ewing Sarcoma: A Case Report

This case report discusses a middle-aged patient with metastatic pleuro-pulmonary Ewing sarcoma, with the rare initial presentation of spontaneous haemothorax. The patient had a prior history of Ewing sarcoma of the pelvis in young age, which had been treated with chemoradiotherapy with remission. There was a 2-week history of right sided chest pain and shortness of breath, and a chest x-ray showed complete opacification of the right hemithorax, with subsequent CT arising suspicion of a heterogeneous soft tissue pleural lesion with fluid in the pleural cavity. While awaiting further treatment including biopsy, the patient deteriorated rapidly and was eventually started on end-of-life care. Postmortem examination showed large right pleural haemorrhage with lung collapse, and histological examination of pleural lesions confirmed a diagnosis of recurrent Ewing's sarcoma. Extraskeletal presentations of Ewing's sarcoma, particularly with spontaneous haemothorax as the initial presentation have been rarely reported in the literature in adult patients. Diagnosing pleural Ewing's sarcoma requires a multimodal approach, utilising investigations such as contrast-enhanced CT imaging, pleural fluid analysis, thoracoscopy, and PET-CT to evaluate for disease spread. Treating pleural Ewing's sarcoma can be challenging; early diagnosis and management of complications such as haemothorax is essential with thoracocentesis or thoracotomy. Ongoing treatment can include multimodal therapies including chemotherapy and radiotherapy with or without surgery, depending on disease extent.