Primary pericardial angiosarcoma with pleural metastasis: an unusual clinical presentation

Abstract

Abstract Introduction/Objective Primary pericardial angiosarcoma is an uncommon, aggressive cardiac neoplasm that arises from the endothelial cells of blood vessels. It typically affects middle-aged men and has a poor prognosis due to early metastasis. Unfortunately, there are currently no established guidelines or effective treatments for this rare malignancy. The purpose of this case study is to emphasize the significance of recognizing the unspecific presentations of pericardial angiosarcoma and ensuring timely diagnosis and appropriate treatment. Methods/Case Report A 56-year-old male presented with recurrent pericardial effusion and exudative pleural effusion. The radiologic studies showed a thickened pericardium with right-sided pericardial effusion and a large right- sided pleural effusion. Resected partial pericardium and parietal pleura showed the presence of epithelioid to spindled cells that formed solid sheets and vascular structures filled with numerous red blood cells. The tumor cells were cytologically malignant, with angulated shapes and hyperchromatic nuclei, and showed extensive invasion into the surrounding connective tissue. Mitotic figures were variably visualized, and there was abundant hemorrhage. Immunohistochemical analysis of the lesional cells showed positivity for ERG, CD31, Factor VIII, and retention of Methylthioadenosine phosphorylase (MTAP) and BRCA1-associated protein 1 (BAP1), while they were negative for WT1, calretinin, claudin-4, and p40. Based on the morphology and immunoprofile, a diagnosis of high grade angiosarcoma was rendered, and the patient underwent chemotherapy. The overall survival of the patient was 8 months to date. Results (if a Case Study enter NA) NA Conclusion Prompt diagnosis of pericardial angiosarcoma is crucial, as nonspecific symptoms may lead to misdiagnosis, such as in this case where the patient was initially diagnosed with pericarditis. Video-assisted thoracic surgery and histologic analysis are necessary to reach a correct diagnosis, which ensures appropriate treatment, as a delayed diagnosis can worsen the prognosis and even lead to death.