Angiosarcoma Causing Cardiac Constriction Late after Radiation Therapy for Breast Carcinoma

Abstract

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

Cardiac Masses in a Patient With Pancreatic Adenocarcinoma and a History of Breast Carcinoma

“…Two cases of postradiation cardiac angiosarcoma have been reported in existing literature. 10 Cardiac sarcomas are lethal tumors, with a reported median survival of around 1 year.…”

“…The most common primary malignant cardiac tumors are sarcomas, with the predominant subtype being angiosarcoma. Two cases of postradiation cardiac angiosarcoma have been reported in existing literature . Cardiac sarcomas are lethal tumors, with a reported median survival of around 1 year.…”

Primary Undifferentiated Pericardial Sarcoma after Radiоtherapy for Hodgkin Lymphoma

“…Only 13 of them were diagnosed with angiosarcoma localized in the mammary gland, anterior chest wall, and skin; the average time of onset was 9.3 years from the moment of radiation exposure. There are 2 cases of pericardial angiosarcoma 8 and 15 years after radiotherapy for breast cancer [ 9 ].…”

“…Patients with some hereditary diseases (tuberous sclerosis, basal-cell nevus syndrome, neurofibromatosis type 1) and after radiation exposure have a higher risk of pericardial sarcoma. Thus, after radiation therapy, 0.017–0.17% of patients are diagnosed with sarcoma (osteosarcoma, angiosarcoma, spindle cell sarcoma) [ 9 ]. Usually, the clinical manifestations of different pericardial tumors depend on its size, location, invasion, and rate of growth but not the origin of pathological process.…”

Metastatic lesion of the right atrium with renal cell carcinoma

“…Однако по информации ряда авторов, включающей в себя заключения эхокардиографии (ЭхоКГ), фиброэластомы являются более распространенными опухолями, чем миксомы [8,9]. Среди злокачественных опухолей наиболее часто встречаются саркомы (40% приходится на ангиосаркомы, которые могли возникнуть вторично после применения лучевой терапии) [10][11][12][13].…”