Welkerling H, Dreyer T, Hermann B, Steiner D, Delling G
Abt. Osteopathologie, Universität Hamburg.
Z Orthop Ihre Grenzgeb. 1990 Nov-Dec;128(6):657-60. doi: 10.1055/s-2008-1040021.
On account of the rarity and its histologic features, the clear-cell chondrosarcoma is a particular entity. Up to the present, 98 cases have been reported in the literature. In the present report one case located in the proximal femur will be discussed. Approximately 60% of these tumors are situated in the mentioned region. Including one referred case, which will not be described in detail, 100 cases have been recognized. Clear-cell chondrosarcoma is radiographically indistinguishable from benign lesions or chondroblastoma. Therefore the histologic examination is crucial for the diagnosis. The histologic characteristics preclude the diagnosis of chondroblastoma. Clear-cell chondrosarcoma is characterized by regions of chondroid matrix and areas of closely packed, glycogen-rich large tumor cells with distinct boundaries. The centrally located nuclei are large with marked polymorphism, surrounded by abundant clear PAS positive cytoplasm. The chondroid matrix is lacking. Osteoid formation and multinucleated giant cells are present in clear-cell areas. In spite of the low malignancy there is still a metastatic potential. Therefore the type of surgical treatment is very important. En bloc excision with wide margins is the treatment of choice. The knowledge about this tumor is crucial for adequate surgical therapy.
鉴于其罕见性及其组织学特征，透明细胞软骨肉瘤是一种特殊的实体。截至目前，文献中已报道98例。在本报告中，将讨论1例位于股骨近端的病例。这些肿瘤中约60%位于上述区域。包括1例引用病例（将不详细描述），已确认100例。透明细胞软骨肉瘤在影像学上与良性病变或软骨母细胞瘤无法区分。因此，组织学检查对诊断至关重要。其组织学特征可排除软骨母细胞瘤的诊断。透明细胞软骨肉瘤的特征是软骨样基质区域以及紧密排列、富含糖原的大肿瘤细胞区域，边界清晰。位于中央的细胞核大，具有明显的多形性，周围是丰富的透明PAS阳性细胞质。缺乏软骨样基质。在透明细胞区域存在类骨质形成和多核巨细胞。尽管恶性程度低，但仍有转移潜能。因此，手术治疗方式非常重要。广泛边缘的整块切除是首选治疗方法。了解这种肿瘤对于充分的手术治疗至关重要。
