儿童遗传性多发性骨软骨瘤软骨帽的超微结构观察  

Ultrastructural features of hereditary multiple osteochondroma cartilage cap in children

作　　者：郭永成[1] 王永堂[1] 陆亚东[1] 夏冰[1] 牛学强[1] 董延召[1] 武慧玲[1] 马润生[1] 

机构地区：[1]郑州大学第三附属医院小儿骨科,郑州450052

出　　处：《临床与实验病理学杂志》2013年第2期171-174,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的观察儿童遗传性多发性骨软骨瘤(hereditary multiple exostoses,HME)软骨帽的超微结构,为儿童HME超微病理诊断提供可靠依据。方法实验组:切除18例HME患儿肋骨瘤体分离软骨帽;对照组:15例胸廓发育畸形患儿手术矫正切除的肋软骨;分别取其纵、横切面应用扫描电镜和透射电镜观察。结果对照组:冷冻断裂的软骨组织内见少量软骨细胞位于软骨陷窝内,软骨组织表面可见大量散乱、稀疏的胶原纤维;软骨细胞数量不多,细胞表面有少量短小的微绒毛,细胞核形状不规则,细胞质内可见到粗面内质网呈条索样分散在细胞质内,线粒体较小,糖原颗粒呈簇状分布。实验组:冷冻断裂的软骨组织内见大量不规则的软骨陷窝,每个软骨陷窝内均含有软骨细胞,细胞表面有丰富的细胞突起;软骨组织内见大量瘤样细胞增生,聚集分布,细胞核较大,细胞质内可见圆形或椭圆形的线粒体及扩张的粗面内质网;瘤细胞间可见毛细血管,其附近可见明显增多的软骨细胞,软骨细胞体积较对照组增大。结论儿童HME软骨帽的超微结构改变(细胞形态及细胞内部细胞器),不同于正常软骨细胞,可能与儿童HME的遗传、发病、发展、转归因素密切相关。Purpose To improve the general understanding of the ultrastructural pathology of children hereditary multiple exostoses （HME） , through the ultrastructural observation of the cartilage cap, and to explore the pathogenesis of the disease to provide a reliable basis for the diagnosis of children HME. Methods Eighteen cases of children HME ribs tumor （experimental group） were reseeted with separating cartilage cap. 15 cases of children thoracic surgical correction were used as control who has costal cartilage developmen- tal malformations. Electron microscopy was used to observe the ultrastructural changes on the longitudinal section and cross section of the tissues, respectively. Results In the control group, the freeze-fracture of the cartilage tissue showed a small amount of cartilage cell located in cartilage lacunae ; a large number of scattered, sparse collagen fibers were observed on the surface of the cartilage tissue ; in a small number of choudrocytes, short microvilli on the cell surface, irregular nucleus, and rough cord-like endoplasmic reticulum, small mitoehondria and glycogen particles were seen within the cytoplasm. In the experimental group, a large number of irregular carti- lage lacunae were seen in the freeze-fracture of the cartilage tissue and each cartilage lacunae contained chondrocytes. It was also ob- served that the ceil surface was covered with plenty of cell protrusions. And a large number of tumor-like cell hyperplasia were found in the cartilage tissue, which had clustered distribution with large nuclei. Round or oval-shaped mitochondria and expansion of rough en- doplasmie reticulum could be seen in the cytoplasm. Capillaries were observed between the tumor cells and cartilage cells, with signifi- cantly increased around the tumor-like cells. Compared with the control group the volume of the cartilage cell appeared considerable large. Conclusions The ultrastructural changes of the children HME cartilage cap （ cell morphology and cell interual organelles ） are different fr

关 键 词：骨肿瘤 遗传性多发性骨软骨瘤 软骨帽 超微结构 儿童 

分 类 号：R726[医药卫生—儿科]