P ericardial constriction remains a difficult diagnosis that requires a high degree of clinical suspicion, careful clinical examination, and multimodality imaging. The cause of the constriction can be challenging to ascertain and often becomes evident only at the time of surgical resection. Such is the case we present.Primary tumors of the pericardium are extremely rare, with an incidence of between 0.001% and 0.28% in an autopsy study 1 ; approximately 8% of those are angiosarcoma. In a study by Kirova and colleagues 2 of 16,705 patients who received radiation therapy (RT) for breast cancer, only 13 patients developed angiosarcoma of the breast, chest wall, or skin after a median follow-up period of 9.3 years. Pericardial angiosarcoma after RT is rare but has been described.1 To our knowledge, this is the 2nd report of angiosarcoma of the pericardium after RT.
Case ReportA 41-year-old woman presented with 8 weeks of progressive dyspnea, orthopnea, and cough. Her history included intracystic papillary carcinoma of the left breast 8 years earlier (treated with lumpectomy and postoperative RT). The details of the protocol and dosage of radiation used during the treatment were not available. The physical examination was consistent with pleural and pericardial effusions. The white cell count was elevated, as were the serum creatinine level (2 mg/dL [normal range, 0.6-1.1 mg/ dL] and the blood urea nitrogen level (46 mg/dL [normal range, 6-21 mg/dL]). Her other laboratory tests-coagulation profile, autoimmune/rheumatologic disease assay, and urinalysis-were within normal limits. A chest radiograph revealed bilateral pleural effusions. Computed tomography (Fig. 1) revealed a large amount of pericardial fluid extending to the aortic root, with an increased density suggesting hemorrhage or high protein content and a possible anterior mass over the right atrium, thought to represent an ill-defined mass or organized hematoma and pleural effusions.Transthoracic echocardiography revealed preserved left ventricular ejection fraction, diastolic dysfunction, bilateral pleural effusions, and evidence of effusive-constrictive pericarditis. Thoracocentesis and pericardiocentesis through a pericardial window revealed no malignant cells. Laboratory evaluation of the effusions for autoimmune and rheumatologic disease was negative.The patient underwent open-chest pericardiectomy for constrictive pericarditis. On the visceral pericardium, there were nodular masses-7-mm thick, rubbery, and dark red-brown-adherent to the parietal pericardium.Microscopically, these were spindle-cell neoplastic cells with marked nuclear pleomorphism, consistent with angiosarcoma (Fig. 2). The malignant cells expressed vimentin, factor VIII, podoplanin, and CD31, with focal expression of CD34. The