摘要目的 探讨心脏原发性炎性肌纤维母细胞瘤(IMT)的临床病理学特点,提高对该病的认识.方法 对4例心脏原发IMT的临床、病理资料进行回顾性分析和随访,进行ALK蛋白的免疫组织化学染色和ALK基因荧光原位杂交检测.结果 4例原发性心脏IMT,男3例,女1例;平均年龄16岁(5个月～30岁);发生于右心室2例,右心房1例,心包1例.组织学类型,肉芽肿型1例,纤维组织细胞型2例,硬化型1例.其中2例ALK蛋白阳性,4例病例均进行ALK融合基因荧光原位杂交检测,未检出阳性病例.结论 心脏IMT非常少见,且临床及组织学表现多样,容易误诊,早期诊断、积极手术是治疗成功的关键;免疫组织化学ALK蛋白阳性表达有助于诊断,ALK基因在IMT中的荧光原位杂交检测目前尚不适宜在临床开展.

abstractsObjective To study the clinical and pathologic features of primary cardiac inflammatory myofibroblastic tumor.Methods A total of 4 patients with primary cardiac inflammatory myofibroblastic tumor were encountered during the period from 1993 to 2013 in National Center for Cardiovascular Disease.The clinical features,imaging findings and outcomes of the 4 patients were evaluated.ALK protein expression and ALK gene status were studied using the archival tumor tissues.Results There were 1 female and 3 male patients.The age of patients ranged from 5 months to 30 years (mean =16 years).The tumor was located in right ventricle (n =2),right atrium (n =1) or pericardium (n =1).Histologic patterns included 2 cases of fibrous histiocytoma type,1 case of granulomatous type and 1 case of sclerosing type.Immunohistochemical study showed that 2 cases expressed ALK protein.Fluorescence in-situ hybridization however did not reveal any ALK gene rearrangement.Conclusions Inflammatory myofibroblastic tumor of the heart is rarely encountered and easily misdiagnosed.It carries distinctive clinical and pathologic features.ALK protein expression is helpful in arriving at the correct diagnosis.