Primary cardiac dedifferentiated liposarcoma in a middle-aged female: a case report

Abstract

Background: Primary malignant cardiac tumors are extremely rare and can present with the same nonspecific characteristics as benign primary cardiac tumors. We herein describe a middle-aged female with an intracavitary, irregular atrial mass who experienced partial surgical resection. The atrial mass which was recognized as myxoma before surgery was finally diagnosed as dedifferentiated liposarcoma (DDLPS) by postoperative pathological examination.

Case presentation: The patient, a 61-year-old female, presented to the emergency room because of progressive chest congestion and shortage of liberties for 6 months and orthopnoea and paroxysmal nocturnal dyspnea for 3 days. The laboratory examinations confirmed no abnormalities. The thoracic computed tomography (CT) scan showed massive hydropericardium, pleural effusion and left atrium occupying lesion. The transesophageal echocardiography (TEE) confirmed an intracavitary and irregular left atrial mass, limiting the mitral valve inflow and pulmonary venous blood reflux. The positron emission tomography/computed tomography (PET/CT) revealed high grade fluorodeoxyglucose uptake only in the intracavitary mass which near the mitral valve. According to operative exploration, the intracavitary mass had invaded the mitral annulus and posterior wall of left ventricle which cannot be resected completely, we did merely partial surgical resection to relieve the patient's symptoms. Postoperative immunohistochemical stain confirmed the diagnosis of DDLPS. The patient was transferred to the oncology department for further therapy. Unfortunately, the patient was detected with brain metastasis 1 month later and died within 5 months after the surgery.

Conclusions: Primary cardiac DDLPS is an extremely rare histological subtype of undifferentiated pleomorphic sarcomas which present the same nonspecific characteristics as benign primary cardiac tumors. Even though surgical resection combined with chemotherapy or radiotherapy remains the mainstream treatment strategy, the prognosis of cardiac malignancy is poor with high mortality. Novel management strategies need to be further explored.

Keywords: Dedifferentiated liposarcoma; Heart failure; Intracavitary atrial mass; Pleural effusion; Primary malignant cardiac tumor.

Fig. 1

Preoperative examinations and intraoperative findings: a CT scans revealed occupying lesion in left atrium and massive hydropericardium and pleural effusion (asterisk and arrows); b The massive right pleural effusion reduced significantly after chest drainage; c TEE showed the left atrial mass limited the mitral valve inflow significantly; d PET/CT confirmed high grade fluorodeoxyglucose uptake only in the intracavitary mass (arrows); e Operative exploration confirmed the atrial mass had invaded the mitral annulus and posterior wall of left ventricle. * The left atrial mass; RV, right ventricle; LV, left ventricle; TEE, Transesophageal echocardiography; PET/CT, Positron emission tomography/computed tomography

Fig. 2

Pathological findings and follow-up imaging examinations: a Markedly atypical cells which lack specific morphological features of differentiation was found by hematoxylin–eosin staining (H&E, 400×); b and c Immunohistochemical staining of the intracavitary mass is strongly positive for MDM2 and CDK4 (Immunostaining, 400×); d Postoperative thoracic CT scan confirmed the mitral obstruction was totally relieved after partial surgical resection (black arrow); e Postoperative brain MRI examination showed the brain metastasis (white arrow)