Key result
Partial resection of cardiac dedifferentiated liposarcoma yields temporary relief with fatal brain metastasis at 5 months.
Why the study?
Primary malignant cardiac tumors are extremely rare and can present with nonspecific characteristics similar to benign cardiac tumors, complicating diagnosis and treatment.
Case Report (n=1)
No
Primary cardiac dedifferentiated liposarcoma is an extremely rare and aggressive malignancy with a poor prognosis despite surgical resection.
Resection offers only transient palliation in cardiac dedifferentiated liposarcoma; leaves open need for multimodal strategies in this rare entity.
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.
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Shen et al. (2019) conducted a case report in Primary cardiac dedifferentiated liposarcoma (n=1). Partial surgical resection was evaluated on Clinical outcome. Partial surgical resection of a primary cardiac dedifferentiated liposarcoma in a 61-year-old female provided temporary relief, but the patient developed brain metastasis and died within 5 months.
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