Primary undifferentiated pleomorphic cardiac sarcoma presenting as right heart failure

Abstract

Right-sided heart failure is a common sequela of left heart failure and seldom presents as a primary disorder. The differential diagnosis of right heart failure includes a cardiac tumor. Cardiac malignancies are rare tumors with an overall poor prognosis. We evaluated a 69-year-old man who presented with a 3-week history of progressive lower extremity swelling, ascites, and scrotal swelling. Laboratory studies were significant only for mildly elevated liver function tests. CT scan of the abdomen and pelvis showed ascites, hepatic swelling, and a bland clot in the inferior vena cava extending from the level of the kidneys to the right atrium. A large mass originating from the right atrium was identified, and biopsy confirmed an undifferentiated pleomorphic cardiac sarcoma. Given the extensive tumor and clot burden, he was not an operative candidate. He developed portal hypertension with esophageal varices and expired due to variceal bleeding.

Keywords: Cardiac sarcoma; Right heart failure; Undifferentiated sarcoma.

Fig. 1

Abdomen/pelvis CT coronal reformat demonstrating thrombus extending from the right atrium (long red arrow) inferiorly to the level of the infra-renal inferior vena cava (short red arrow). Dense accumulation of intravenous contrast within the right atrium.

Fig. 2

Abdomen/pelvis CT with contrast showing right atrial sarcoma extending into inferior vena cava (red arrow).

Fig. 3

Abdomen/pelvis CT coronal MIPS reformat demonstrating thrombus at the origin of the left renal vein (red arrow), contiguous with the IVC thrombosis.

Fig. 4

TTE showing a large, calcified, circular mass in the right atrium (red arrow) with an associated thickened pericardium.

Fig. 5

High magnification microscopic histopathology with H&E staining revealed the presence of medium-sized hypochromatic spindled nuclei without prominent nucleoli and moderate eosinophilic cytoplasm with indistinct cell borders.