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间充质和肌肉特异性中间丝(波形蛋白和结蛋白)与儿童横纹肌肉瘤分化的关系。

Mesenchymal and muscle-specific intermediate filaments (vimentin and desmin) in relation to differentiation in childhood rhabdomyosarcomas.

作者信息

Molenaar W M, Oosterhuis J W, Oosterhuis A M, Ramaekers F C

出版信息

Hum Pathol. 1985 Aug;16(8):838-43. doi: 10.1016/s0046-8177(85)80256-2.

Abstract

Twenty-one childhood rhabdomyosarcomas were divided into three groups on the basis of cytologic composition. The tumors in group P consisted entirely of primitive mesenchymal cells, whereas those in groups M and W were characterized by the additional presence of numerous round rhabdomyoblasts and strap cells, respectively. The tumors were studied for the universal mesenchymal intermediate filament vimentin, and for the muscle-specific intermediate filament desmin. Vimentin positivity, which tended to be more prominent in primitive tumor cells, was found in all tumors, whereas desmin was found especially in round rhabdomyoblasts and strap cells. Desmin-positive primitive cells were found only in groups M and W, not in group P. It was concluded that the differentiation from primitive mesenchymal cells to morphologically recognizable myogenic tumor cells is accompanied by an increase in desmin positivity and, presumably, a decrease in vimentin positivity. Moreover, the observations suggest the existence of a group of "committed" cells that are morphologically primitive, but desmin-positive. These cells might play an important role in the observed further differentiation of rhabdomyosarcomas under chemotherapy.

摘要

21例儿童横纹肌肉瘤根据细胞组成分为三组。P组肿瘤完全由原始间充质细胞组成,而M组和W组肿瘤的特征分别是额外存在大量圆形横纹肌母细胞和带状细胞。对这些肿瘤进行了通用间充质中间丝波形蛋白以及肌肉特异性中间丝结蛋白的研究。波形蛋白阳性在所有肿瘤中均有发现,在原始肿瘤细胞中往往更为突出,而结蛋白尤其在圆形横纹肌母细胞和带状细胞中发现。仅在M组和W组中发现了结蛋白阳性的原始细胞,P组中未发现。结论是,从原始间充质细胞向形态上可识别的成肌肿瘤细胞的分化伴随着结蛋白阳性的增加,并且推测波形蛋白阳性降低。此外,这些观察结果提示存在一组形态上原始但结蛋白阳性的“定向”细胞。这些细胞可能在化疗下观察到的横纹肌肉瘤进一步分化中起重要作用。

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