RECURRENT PLEURAL AND PERICARDIAL EFFUSIONS IN A PATIENT WITH A RIGHT ATRIAL ANGIOSARCOMA: A CASE REPORT

• Published in: Anticancer research Vol. 38; no. 3; p. 1831
• Main Authors: Carroll, Kristen, Kloefkorn, Chad, Zatakia, Jigna
• Format: Journal Article
• Language: English
• Published: Athens International Institute of Anticancer Research 01.03.2018
• Subjects: Anticoagulants; Atrium; Biomarkers; Biopsy; Case reports; Cellular biology; Colchicine; Collagen; Computed tomography; Coronary artery disease; Cough; Cytology; Diagnosis; Diagnostic systems; Differential diagnosis; Edema; Effusion; Embolism; Epithelial cells; Evacuation routing; Exudation; Heart diseases; Heart rate; Laparoscopy; Lesions; Liver; Lung diseases; Magnetic resonance imaging; Malignancy; Markers; Medical imaging; Medical prognosis; Metastases; NMR; Nuclear magnetic resonance; Pain; Pathology; Patients; Pericardium; Skin; Spleen; Steroids; Surgery; Survival; Tumors; Vascular diseases
• ISSN: 0250-7005; 1791-7530

Background: Angiosarcomas are rare endothelial vascular malignancies composed of anaplastic epithelial cells. These aggressive tumors have wide metastatic potential via both hematogenous and lymphatic spread. They tend to occur between the third and fifth decades of life, more frequently in men. Although most commonly originating in soft tissue and skin, angiosarcomas can develop from the pericardium, liver, spleen or even breast. Angiosarcomas represent the most common type of primary cardiac malignancy but overall are extremely rare and often fatal. Methods: We present a case of a 36-year-old previously healthy male who initially presented with bilateral lower extremity edema and was found to have large recurrent pericardial effusions. The pericardial fluid was predominately bloody with scant cellularity, cultures were negative, and cytology did not reveal malignant cells. Pericardial biopsy showed nonspecific chronic inflammation. His effusion was thought to be due to post-infectious immune-mediated pericarditis, for which he was given pulse dose steroids and discharged on colchicine. Four months later he presented with subacute cough and was found to have a large left-sided blood exudative pleural effusion, again with negative cultures and cytology, eventually requiring pleurodesis. An extensive rheumatologic work-up, tumor markers, pleural and lung biopsies were non-diagnostic. MRI revealed a right atrial irregularity thought to be a clot and further imaging showed multiple small hypodense liver lesions. The patient was discharged on anticoagulation, but was lost to follow-up, only to return two months later with abdominal pain and hyperbilirubinemia. Imaging revealed multiple liver masses with extravasation suggesting hepatic metastases for which he underwent laparoscopy with hemoperitoneum evacuation. Surgical pathology was consistent with angiosarcoma involving the pericardium, right middle lung and liver, most likely of primary right atrial or pericardial origin. Results: Although rare, the majority of cardiac angiosarcomas originates in the right atrium and can invade local structures, including the pericardium. Patients typically present late after significant tumor progression because the initial symptoms are nonspecific and are frequently misdiagnosed as acute pericarditis. Conclusion: The differential diagnosis for recurrent exudative pleural and pericardial effusions is short and includes infection, pulmonary embolism, collagen vascular disease and malignancy, notably cardiac angiosarcomas. Patients with cardiac angiosarcomas exhibit rapid progression of symptoms and advanced clinical deterioration. Prognosis for angiosarcoma is very poor: mean survival is estimated at 6-14 month with a 5-year survival rate of only 14%. Cardiac magnetic resonance imaging is better than computed tomography in identifying an atrial abnormality, but still cannot reliably differentiate a thrombus from a tumor, thus diagnosis frequently requires surgical pathology. The absence of malignant cells on fluid cytology, normal pericardial/pleural biopsies, and negative tumor markers cannot rule out an angiosarcoma.