目的　探讨低分化心内膜肉瘤的诊断与治疗。方法　回顾分析1例低分化心内膜肉瘤患儿的临床资料。结 果　患儿，女， 1岁，因腹泻、气促、发绀和咳嗽入院。听诊有异常心音。心电图示Ⅱ、Ⅲ、aVF导联P波高尖。经超声心动 图和心脏术后病理检查确诊为低分化心内膜肉瘤。出院3个月后患儿在家中突然出现昏迷并死亡。结论　低分化心内膜 肉瘤的诊断主要依据临床表现、脏超声心动图及病理学检查，治疗以外科手术切除为首选，化疗和放疗有辅助治疗作用， 但目前无治愈方法。

　Objective　To explore the diagnosis and treatment of poorly differentiated endocardial sarcoma. Method　The clinical data of a child with poorly differentiated endocardial sarcoma was retrospectively analyzed. Results　One-year-old girl was admitted for diarrhea, polypnea, cyanosis, and cough. Abnormal heart sound was found by auscultation. Leads Ⅱ, Ⅲ, and aVF of ECG showed high peaked P wave. The diagnosis of poorly differentiated endocardial sarcoma was confirmed by echocardiography and pathology after cardiac operation. Three months after discharge from the hospital, the patient suddenly came into coma and died. Conclusion　The diagnosis of poorly differentiated endocardial sarcoma is mainly based on clinical manifestations, echocardiography and pathology. Surgical resection is the first choice and chemotherapy and radiotherapy play a supporting role. However, there is no cure for it currently.