Histopathologic and clinical characterization of cardiac myxoma: Review of 53 cases from a single institution

Histopathologic and clinical characterization of cardiac myxoma: Review of 53 cases from a single institution
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DOI:
10.1067/mhj.2000.107176
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发表时间:
2000-07-01
影响因子:
4.8
通讯作者:
Mollo, F
Mollo, F
中科院分区:
医学2区
文献类型:
--
作者:
Pucci, A;Gagliardotto, P;Mollo, F

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背景心脏粘液瘤具有不同的临床表现、不确定的组织发生和有争议的免疫组织化学特征。此前已有少数恶性病例报道。方法对连续53例心脏粘液瘤进行组织学检查,并将结果与​​临床资料进行比较。该研究的主要目标是调查免疫组织化学分化和临床病理相关性。 结果 基质细胞的特征是血管性血友病因子内皮标记物(53 例中的 12 例)和弥漫性细胞质神经肽的表达,例如蛋白质基因产物 9.5(53 例中的 50 例)、S100 蛋白(53 例中的 47 例)和神经元特异性烯醇化酶(53 例中的 30 例),所有这些都在 53 个肿瘤中的 30 个(57%)中表达。基质细胞不显示内分泌颗粒、上皮或平滑肌免疫反应性。 53 例患者中有 7 例观察到非心脏相关症状,并在肿瘤切除后迅速消失;这7例中神经肽总免疫反应性评分的中值和百分比较高,但数据分析显示无统计学意义。 2 例粘液瘤中检测到腺体,并显示上皮(细胞角蛋白和癌胚抗原)、蛋白 S100 和神经元特异性烯醇化酶免疫反应性;先前已在人类肠道中检测到这种模式。所有肿瘤均表现出良性行为,未检测到有丝分裂。结论本研究结果支持基质细胞起源于能够神经和内皮分化的多能间充质的假设;罕见的粘液瘤腺体代表被困的前肠休息。神经内分泌分化和非心脏相关症状之间可能存在相关性。
Background Cardiac myxomas have varying clinical presentation, uncertain histogenesis, and debatable immunohistochemical profile. A few malignant cases have been previously reported.Methods Fifty-three consecutive cardiac myxomas were histologically investigated and results compared with clinical data. The main goal of the study was to investigate the immunohistochemical differentiation and the clinicopathologic correlations.Results Stromal cells were characterized by the expression of the von Willebrand factor endothelial marker (12 of 53 cases) and diffuse cytoplasmic neuropeptides such as protein gene product 9.5 (50 of 53 cases), S100 protein (47 of 53) and neuron-specific enolase (30 of 53), all of which were expressed in 30 (57%) of 53 tumors. Stromal cells did not show endocrine granules, epithelial, or smooth muscle immunoreactivity. Non-cardiac-related symptoms were observed in 7 of 53 patients and promptly disappeared after tumor excision; median values and percentages of total immunoreactivity scores for neuropeptides were higher in these 7 cases, but data analysis showed no statistical significance. Glands were detected in 2 myxomas, and they showed epithelial (cytokeratins and carcinoembryonic antigen), protein S100, and neuron-specific enolase immunoreactivity; this pattern has been previously detected in human gut. All tumors showed benign behavior, and no mitosis was detected.Conclusions The results of this study support the hypothesis that stromal cells originate from multipotent mesenchyme capable of neural and endothelial differentiation; rare myxoma glands would represent entrapped foregut rests. A correlation could exist between neuroendocrine differentiation and non-cardiac-related symptoms.