New-onset heart failure masking a massive retroperitoneal liposarcoma

Abstract

L iposarcoma is one of the most common soft tissue sarcomas, representing 50% of all retroperitoneal sarcomas. A large retroperitoneal sarcoma can exert mass effect on intraabdominal organs and the thoracic cavity producing symptoms similar to those seen in acute decompensated heart failure (HF), such as abdominal swelling, dyspnea on exertion, and orthopnea. However, the presentation with new-onset HF masking a massive retroperitoneal liposarcoma is exceedingly rare. Here, we present an interesting case of an elderly patient with new-onset decompensated HF in the setting of multi-vessel coronary artery disease who responded well to intravenous diuretic therapy without complete symptom resolution. Further workup showed a massive retroperitoneal sarcoma as the contributing etiology. Given his poor cardiac function, he was not initially a surgical candidate. However, after being placed on appropriate goal-directed medical therapy, he experienced recovery in his ejection fraction (EF) and is now a surgical candidate. A 78-year-old male with a history of hypertension, hyperlipidemia, and tobacco abuse came to the emergency room with complaints of progressive shortness of breath, orthopnea, and abdominal distention. On initial evaluation, the patient was normotensive, tachycardiac, and volume overloaded. The laboratory results showed elevated N-terminal pro-B-type natriuretic peptide level with a benign comprehensive metabolic panel. Chest X-ray revealed cardiomegaly with pulmonary vascular congestion. Transthoracic echocardiogram showed a severely reduced EF of 10%–15%, left ventricular eccentric hypertrophy and global hypokinesis. He responded well to intravenous diuretics, but had persistent orthopnea and minimal improvement of abdominal distension. He underwent computed tomography of the abdomen and pelvis which showed an extremely bulky, multi-lobulated retroperitoneal mass measuring 29 cm × 33 cm × 35 cm with mass effect on multiple intra-abdominal organs (Figure 1). Computed tomography guided core needle biopsy of the mass confirmed well-differentiated liposarcoma (WDLPS) with atypical spindle cells and adipocytes (Figure 2). Fluorescence in situ hybridization analysis revealed murine double minute 2 (MDM2) amplification. No evidence of metastatic disease was found on additional workup. The patient was not initially a candidate for surgical resection due to his low EF, however, after three months of goal-directed medical therapy, he had recovery in EF from 10%− 15% to 40%. Outpatient ischemic evaluation revealed multivessel disease and he underwent coronary artery bypass surgery. He is planned for tumor resection. Sarcomas are a heterogeneous group of over 80 different tumors arising from mesenchymal or connective tissue. In 2018, soft tissue sarcomas represented approximately 0.8% of all cancers in the United States. Pure WDLS accounts for approximately Journal of Geriatric Cardiology