4例原发性心脏横纹肌瘤临床病理分析

目的:探讨原发性心脏横纹肌瘤的临床病理特点、诊断和鉴别诊断、治疗及预后特点。方法:对3例胎儿尸检病例及1例婴儿手术切除病例行肉眼及石蜡切片观察,并做免疫组化及特殊染色。结果:4例患儿均为心脏横纹肌瘤,其中1例胎儿尸检伴发大脑皮质内多发结节,诊断为结节性硬化症,肿瘤细胞Myoglobin(+)、Desmin(+)、Vimentin(+);脑部结节S-100(+)、GFAP(+)、Nestin(+)。结论:心脏横纹肌瘤好发于胎儿及5岁的婴幼儿,其合并结节性硬化症者比例较低,免疫组织化学标记和特殊染色有助于肿瘤的诊断和鉴别诊断。

Clinicopathology analysis of 4 cases in primary cardiac rhabdomyomas

Objective：To explore the morphological features, diagnosis, differential diagnosis, therapy and prognosis of the primary cardiac rhabdomyomas. Methods ： Clinicopathology data of 4 cases of the primary cardiac rhabdomyomas were analyzed, and observed by macroscopy and microscopy. Paraffin-embedded tissue section were done with immunohistochemistry and specific staining. Results： All of the four cases were diagnosed as the primary cardiac rhabdomyomas, in addition one of them companied by brain parenchymal multiple nodus. The tumor cells were positive in myoglobin, Desmin and Vimcntin, meanwhile the node in the brain expressed S-100 ,GFAP and Nestin. Conclusion：Cardiac rhabdomyomas typically occurs in infants. The incidence of cardiac rhabdomyomas associated with tuberous sclerosis is lower. Marks of immunohistochemistry and special histochemical staining could help the diagnosis.