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新生儿肺横纹肌瘤病:一例伴有肺内横纹肌细胞免疫组化及电镜特征的病例报告

Rhabdomyomatosis of newborn lung: a case report with immunohistochemical and electronmicroscopic characterization of striated muscle cells in the lung.

作者信息

Chen M F, Onerheim R, Wang N S, Hüttner I

机构信息

Department of Pathology, McGill University, Montreal, Quebec, Canada.

出版信息

Pediatr Pathol. 1991 Jan-Feb;11(1):123-9. doi: 10.3109/15513819109064748.

DOI:10.3109/15513819109064748
PMID:2014187
Abstract

Interstitial proliferation of striated muscle cells in the lung is rare. A few cases in the literature only document infants with associated lung and other major organ anomalies incompatible with long-term survival. In this report we document a case of diffuse patchy interstitial proliferation of striated muscle cells in the left lung and confirm their nature by immunohistochemistry and electron microscopy. In particular, we show that these skeletal muscle cells express alpha-sarcomeric actin, but not alpha-smooth muscle actin, indicating an antigenetically well-developed striated muscle phenotype despite morphologically embryonal features.

摘要

肺内横纹肌细胞的间质增生较为罕见。文献中仅有少数病例记载了伴有肺部及其他主要器官异常且无法长期存活的婴儿。在本报告中,我们记录了一例左肺横纹肌细胞弥漫性斑片状间质增生的病例,并通过免疫组织化学和电子显微镜证实了其性质。特别是,我们发现这些骨骼肌细胞表达α-肌节肌动蛋白,但不表达α-平滑肌肌动蛋白,这表明尽管在形态上具有胚胎特征,但在抗原方面具有发育良好的横纹肌表型。

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