左心房原发性黏液瘤14例临床病理分析

Clinicopathologic features of primary left atrial myxoma:an analysis of 14 cases

目的分析左心房原发性黏液瘤的临床特征、病理学特点,探讨其组织来源、诊断及鉴别诊断。方法收集14例心脏黏液瘤患者的临床资料,对组织病理学表现和免疫组化结果进行分析,并结合文献复习。结果 14例心脏黏液瘤患者,男性3例,女性11例,年龄20～64岁,平均年龄50.2岁;黏液瘤均位于左心房。除1例没有临床症状外其余均有胸闷、心悸、气促。镜下大量黏液样基质中可见散在或灶状分布的肿瘤细胞,细胞呈星芒状、梭形、圆形或卵圆形,核深染,局部可见多核瘤细胞。免疫组化:14例vimentin均(+),SMA灶性(+),Ki-67(-);6例calretinin灶性(+)。结论左心房黏液瘤可能起源于原始多潜能间叶细胞,可向平滑肌分化;但部分细胞calretinin(+)。组织病理结合超声心动图可明确诊断。该瘤复发率较低,预后较好。

Objective To study the clinical and pathological features,origin of tissue,diagnosis,differential diagnosis and prognosis of left atrial myxoma.Methods The clinical data,histopathological features and the result of immunohistochemical staining were summarized in 14 cases of cardiac myxoma with review of the relative literatures.Results There were 3 males and 11 females.The youngest was 20 years and the oldest was 64 years.The mean age was 50.2 years.All the tumors were in the left atrium.All the others had clinical symptoms except one.The main symptoms were chest distress,palpitation and anhelation.On microscopy,myxoma was made up of mucoid stroma with variable myxoma cells.The cells may be stellate,spindle,round or oval with deep stained nuclei.Sometimes polynuclear neoplastic cells could be seen.Immunohistochemically,tumor cells were diffusely positive for Vimentin（14 cases）,local positive for SMA（14 cases） and Calretin in（6 cases）,negative for Ki67（14 cases）.Conclusions The clinical manifestation of left atrial myxoma is diverse and the symptom of mitral stenosis or obstruction is frequently showed.The diagnosis needs a combination of echocardiogram and histopathological examination.The tumor may originate from primitive multipotential mesenchymal cells,capable of smooth muscle differentiation;but Calretinin is expreesed in some cells.The recurrence rate is low and the prognosis is good.