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低分化横纹肌肉瘤的超微结构:一例报告及文献复习

The ultrastructure of poorly differentiated rhabdomyosarcomas: a case report and literature review.

作者信息

Bundtzen J L, Norback D H

出版信息

Hum Pathol. 1982 Apr;13(4):301-13. doi: 10.1016/s0046-8177(82)80220-7.

Abstract

The ultrastructure of a case of rhabdomyosarcoma is reported, and the world literature on the ultrastructure of rhabdomyosarcomas is reviewed. The reported case was first observed as an undifferentiated malignancy originating in the maxillary antrum of a 46-year-old white woman and later showed differentiated cells in metastatic sites. A specific feature of the sarcomere within a representative malignant cell is necessary and sufficient for diagnosis of rhabdomyosarcoma by ultrastructure. Examples of specific features include thick and thin filaments in a hexagonal array, an unambiguous Z line, or A bands with H and M bands. The presence of thick and thin filaments without additional features of the sarcomere indicate a myogenous origin, either leiomyosarcoma or rhabdomyosarcoma. Other ultrastructural features observed in rhabdomyosarcomas, for which the diagnosis is confirmed by the presence of diagnostic ultrastructural features or by the presence of cross-striations observed by light microscopy, include large irregularly shaped nuclei with projections and invaginations, free ribosomes closely associated with filaments, rough endoplasmic reticulum that is often dilated, glycogen, and external lamina, prominent nucleoli, and pleomorphic mitochondria. When this spectrum of features is present, the possibility of rhabdomyosarcoma cannot be excluded, and a diligent search for the diagnostic components of the sarcomere should be made. Small intercellular junctions, pinocytotic vesicles, and paranuclear cilia occurred in the poorly differentiated metastasis of this case; the presence of these nonspecific features permits neither diagnosis nor exclusion of rhabdomyosarcoma.

摘要

本文报告了一例横纹肌肉瘤的超微结构,并对横纹肌肉瘤超微结构的世界文献进行了综述。所报告的病例最初表现为起源于一名46岁白人女性上颌窦的未分化恶性肿瘤,后来在转移部位出现了分化细胞。代表性恶性细胞内肌节的特定特征对于通过超微结构诊断横纹肌肉瘤是必要且充分的。特定特征的例子包括呈六边形排列的粗细肌丝、明确的Z线或带有H带和M带的A带。仅存在粗细肌丝而无肌节的其他特征表明为肌源性起源,可能是平滑肌肉瘤或横纹肌肉瘤。在横纹肌肉瘤中观察到的其他超微结构特征,其诊断可通过存在诊断性超微结构特征或通过光学显微镜观察到的横纹来证实,包括具有突起和内陷的大的不规则形细胞核、与肌丝紧密相关的游离核糖体、常扩张的粗面内质网、糖原和外板、明显的核仁以及多形性线粒体。当出现这些特征谱时,不能排除横纹肌肉瘤的可能性,应仔细寻找肌节的诊断成分。在该病例的低分化转移灶中出现了小的细胞间连接、胞饮小泡和核旁纤毛;这些非特异性特征的存在既不能确诊也不能排除横纹肌肉瘤。

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