One in One Million—A Case of Pleural Disease

This case report presents an instance of pleural epithelioid hemangioendothelioma (EHE), a vascular tumour with an incidence of less than 1% among vascular tumors. The patient, a 43-year-old man, presented with a right-sided pleural effusion, longstanding neck and shoulder pain, and worsening pleuritic chest pain. Initial imaging revealed a left infra-clavicular soft tissue mass, pleural thickening, and pulmonary nodules suggestive of metastases. Despite inconclusive initial biopsies, immunohistochemistry and an international pathology review confirmed EHE, characterized by CAMTA1 expression and WWTR1 CAMTA1 fusion. The pleural involvement indicated metastatic disease, leading to a poor prognosis. Treatment with the MEK inhibitor trametinib was initiated, but the patient died within 3 months. This case underscores the diagnostic challenges of EHE due to its rarity and variable clinical presentation, which often delays diagnosis until advanced stages. The report highlights the aggressive nature of pleural EHE and the lack of standardized treatments, emphasizing the need for early recognition.