INTRODUCTION: Acute liver failure refers to the rapid development of severe acute liver injury with impaired synthetic function and encephalopathy in a person who previously had a normal liver or had well-compensated liver disease. In the United States, the leading cause is acetaminophen ingestion (39 percent), indeterminate (17 percent), idiosyncratic drug reactions (13 percent), and viral hepatitis A or B (12 percent). Budd-Chiari syndrome is a relatively uncommon cause of acute liver failure. Primary malignant tumors of the heart are an exceedingly rare cause of this syndrome . We present a case of a patient that presented with fulminant hepatic failure (FHF) secondary to a cardiac synovial sarcoma that induced a Budd-Chiari syndrome.
CASE PRESENTATION: A 60 year old Caucasian male with history of hypertension and Parkinson’s disease who developed progressive lower extremity edema, was transferred from an outside facility with acute liver failure of unknown etiology. The patient had bilateral lower extremity edema, ascites, was initially anicteric and had no stigmata of chronic liver disease. Subsequent workup was unrevealing for common causes of FHF. Bilateral lower extremity Doppler ultrasonography showed deep venous thrombosis of the entire femoral-popliteal system bilaterally with extension into both iliac veins. Carbon dioxide (CO2) gas was used to perform an inferior vena cava venogram. It demonstrated extensive thrombus at the level of the inferior vena cava and it was not feasible to place the IVC filter. There was evidence of lack of flow into the right atrium suggesting hepatic vein thrombosis during a CO2 contrasted angiography. Liver ultrasonography demonstrated hepatic vein thrombosis and reversal of portal venous flow. A computed tomography study demonstrated extension of thrombus to the right atrium with suggestion of a mass. Echocardiography revealed a large echo-dense mass in the right atrium. A magnetic resonance imaging (MRI) study confirmed a 10.6 x 5.0 x 5.6 cm heterogeneous mass within the pericardium, adjacent to the right atrium and invading this same chamber (Figure 1). Our initial differential diagnosis included an angiosarcoma, sarcoma, hepatocellular carcinoma, and thrombus. Cardiothoracic surgery evaluation was performed, but given the extent of invasion of the tumor it was deemed that intervention would carry a high chance of mortality. Furthermore, given the morbidity associated with simultaneous liver transplantation, it was decided with the patient’s wife that it was best to proceed pursuing comfort measures only. Autopsy revealed a large mass originating in the lateral wall of the right atrium that on histological examination was consistent with a highly differentiated synovial sarcoma. Metastatic tumor nodules in the visceral and parietal pericardium were identified but not elsewhere. There was confirmation of extensive clot extending from the hepatic veins to the right atrium that is consistent with a Budd-Chiari syndrome.
DISCUSSION: Primary cardiac neoplasm is a rare entity with an estimated frequency of 0.0017- 0.33% . Seventy-five percent of cardiac masses are benign and 25% are malignant, of which 95% are sarcomas and 5% are primary lymphomas. Synovial sarcomas account for approximately 5% of all cardiac tumors.  The prognosis for cardiac sarcomas remains dismal because of the advanced tumor stage at presentation.. Only fourteen cases of primary cardiac synovial sarcomas are reported in the literature (12 men, 2 women) with a median age of 37 years (range of 13 to 53 years), with none of these presenting with acute liver failure .
CONCLUSIONS: To our knowledge, this represents the first reported case of a rare primary cardiac synovial sarcoma presenting unusually as a less common cause of FHF, Budd-Chiari syndrome.
Reference #1 Boulmay B, Cooper G, Reith JD, Marsh R. Primary cardiac synovial sarcoma: A case report and brief review of literature. Sarcoma 2007;10.1155:94797.
Reference #2 Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004;350(6):578.
Reference #3 Mayer F, Aebert H, Rudert M, Kφnigsrainer A, Horger M, Kanz L. Primary malignant sarcomas of the heart and great vessels in adult patients: A single centre experience. Oncologist 2007;12:1134-42.
DISCLOSURE: The following authors have nothing to disclose: Jorge Guerrero, Jonathan Stine, Caitlin Crowther, Kelly Newton, Ajeet Vinayak
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