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目的:探讨正常的软骨内化骨障碍导致恶变现象的原因。方法:对Ollier病合并Maffucci综合征进行HE染色观察,并复习文献。结果:由骨髓组织和薄层梁状骨包绕的软骨岛可见不同发育时期、形态较为异常的软骨构成,这些发育异常的软骨细胞是正常软骨化骨缺陷,并非真性肿瘤。组织学上具特征性的Ollier病合并Maffucci综合征软骨细胞呈圆形、卵圆形,胞浆丰富,内含大小不等空泡,核圆形位于中央,染色质深。细胞可见淡蓝色,均质的软骨基质。黏液样基质中可见部分软骨细胞呈梭形或多角形,并可见此软骨细胞移行过渡至较成熟软骨细胞的现象。症状发生变化及病变扩展至骨皮质外的周围软组织预示已经进展为软骨肉瘤。结论:Ollier病或Maffucci综合征等软骨病变可能为系统性中胚叶发育异常的先天性肿瘤综合征。
Objective: To investigate the causes of malignant transformation of normal endochondral cartilage disorders. Methods: Ollier’s disease with Maffucci syndrome was observed by HE staining and the literature was reviewed. Results: The cartilaginous island surrounded by myeloid tissue and thin beam bone showed that cartilage was abnormal in different developmental stages and morphology. These dysplastic chondrocytes were normal cartilage defects and were not true tumors. Histologically characterized Ollier’s disease combined with Maffucci syndrome Chondrocytes were round, oval, abundant cytoplasm, containing vacuoles ranging in size from the central round nucleus, deep chromatin. Cells visible light blue, homogeneous cartilage matrix. Some of the chondrocytes in the mucus-like matrix were spindle or polygonal, and the migration of chondrocytes to mature chondrocytes was seen. Symptoms and pathological changes to extend beyond the cortical extracranial soft tissue has progressed to chondrosarcoma. Conclusion: Cartilage diseases such as Ollier’s disease or Maffucci’s syndrome may be congenital neoplasms with systemic mesoblobular dysplasia.