Giant primary cardiac liposarcoma vascularized via the circumflex coronary artery

Abstract

A 51-year-old patient presented with a history of chest pain and progressive dyspnea. CT scan demonstrated a lipomatous mass encircling the heart and compressing the left lung. After median sternotomy, an intrapericardial tumor of 3200 g with a vascular pedicle arising from the circumflex coronary artery was resected. A histologic diagnosis of a well-differentiated liposarcoma was made. The patient is alive and well 2 years postoperatively. This tumor with its unusual vascularization is the largest of the few primary cardiac liposarcomas that have been reported.

Undiagnosed Primary Cardiac Liposarcoma in an Adult

“…7 The frequency of primary heart mesenchymal tumors found during autopsies is estimated as high as 0.0017% to 0.28% and most of them (approximately 75%) are benignVmyxomas, rhabdomyomas, lipomas, and fibromas. 3 Liposarcomas malignant potential varies greatly according to its different histologic classes. By contrast, metastatic involvement of the heart and pericardium is far more common (20Y40 times), 4 occurring in 1.5% to 21% of all autopsies performed on patients.…”

“…2 Atypical lipomatous tumors/well-differentiated liposarcomas are composed entirely or in part of an adipocytic proliferation (both lipoblasts and fat cells), showing significant variation in cell size, and at least focal nuclear atypia in both adipocytes and stromal cells. 3 Round cell and pleomorphic liposarcomas are aggressive tumors that are characterized by infiltrative growth, early metastasis to the lungs, and frequent local recurrence after surgical resection. 3 Liposarcomas malignant potential varies greatly according to its different histologic classes.…”

“…They primarily occur in the retroperitoneum, buttocks, vulva, neck, and mediastinum, usually recur locally and rarely metastasize to distant sites including the heart and pericardium. 3 The diagnosis of cardiac involvement is seldom made antemortem because small lesions are usually silent as in not helpful in achieving a diagnosis. 3 The diagnosis of cardiac involvement is seldom made antemortem because small lesions are usually silent as in not helpful in achieving a diagnosis.…”

“…2 The incidence of primary cardiac liposarcomas is extremely low as well. 3 If they remain undiagnosed, they may lead to sudden death, due to congestive heart failure or cardiac tamponade. 3 If they remain undiagnosed, they may lead to sudden death, due to congestive heart failure or cardiac tamponade.…”

Left ventricular lipoma with pseudoaneurysm-like appearance

“…3 Complete tumor resection was essential to obtain a good prognosis regardless of its pathological fi ndings. 4 We dissected and completely enucleated the elastic mass with a safe surgical margin and preserved the normal left ventricle muscle structure. After placing 10 transmural interrupted horizontal mattress 3-0 nonabsorbable monofi lament sutures with small Tefl on felt pledgets, a composite patchround equine pericardial patch lined with expanded polytetrafl uoroethylene (ePTFE) cardiovascular patchwas sutured into place to maintain the oval shape of the left ventricle.…”