透明细胞软骨肉瘤临床病理诊断  

Clinicopathologic diagnosis of clear cell chondrosarcoma

作　　者：张惠箴[1] 蒋智铭[1] 朱雄增[2] 施琳[1] 陈洁晴[1] 黄瑾[1] 韩蕴华[1] 

机构地区：[1]上海交通大学附属第六人民医院病理科,上海2002331 [2]复旦大学附属肿瘤医院病理科,上海200032

出　　处：《临床与实验病理学杂志》2007年第3期317-320,共4页Chinese Journal of Clinical and Experimental Pathology

基　　金：上海市卫生局科技发展基金资助项目(034042)

摘　　要：目的探讨透明细胞软骨肉瘤的临床、影像学、病理学、生物学行为等特征以及病理鉴别诊断。方法报道4例透明细胞软骨肉瘤，对其临床、病理和随访资料作系统整理，并行免疫组化EnVision两步法S-100蛋白、Col Ⅱ和其它与鉴别诊断相关的蛋白标记。结果4例都是中老年男性，均位于长骨骨端，病程较长。X线表现为纯溶骨性骨质破坏，早期边界清楚，后期病灶扩大并向骨干方向延伸，但均未穿透骨皮质，不形成骨外软组织肿块。大体肿瘤灰白色，有弹性，其中2例伴动脉瘤性骨囊肿。镜下瘤细胞边界清楚，有分叶结构，胞质丰富透明或嗜酸性毛玻璃状。透明细胞之间有反应性新骨和破骨细胞样巨细胞增生。其中3例含低级别普通软骨肉瘤成分。免疫组化标记S-100蛋白和Col Ⅱ阳性。临床治疗和随访：例1行右股骨下端病灶刮除后6年原位复发，再作病骨切除和人工膝关节置换。例2行股骨颈肿瘤切除后植骨，缝匠肌骨瓣移植及内固定术，术后放疗。例3行股骨下端肿瘤切除后骨水泥填充，术后放疗。上述3例经2年4个月～7年随访情况均良好。例4行右股骨上端截除手术，术后失访。结论透明细胞软骨肉瘤是一种罕见的低度恶性软骨肉瘤，有不同于普通软骨肉瘤的临床、影像学和病理形态学特点。在病理诊断时既应注意与良性软骨母细胞瘤和骨母细胞瘤鉴别，也应与高度恶性透明细胞型骨肉瘤和转移性透明细胞癌鉴别。Purpose To investigate characteristics of clear cell chondrosarcoma （CCCS） in clinicopathology, imaging, biological behavior, and differential diagnosis. Methods There were four cases of clear cell chondrosarcoma, and their clinical data, pathologic record, and follow - up materials were collected and reviewed. Immunohistochemical stains were applied for differentiation. Results All patients were middle - aged to elderly male with long duration. All lesions were located at the epiphyses of the tubular bone. X-ray showed a bone lytic defect with sharp demarcation in the early phase and expansion progressing toward diaphyses in the late phase. The lesion did not penetrate the cortex, and there was no extraskeletal soft tissue mass. Grossly they appeared grayish white. Two of them had coexistent aneurysmal bone cysts. Microscopically, clear cells arranged in an indistinct lobular pattern characterized the tumor. The centers of the lobules contained short trabeculae of bone and osteoclast-like giant cell intermingled with the cells between the lobules. Tumor cells were well delineated with clear and vaculated cytoplasm. S-100 protein and type Ⅱcollagen were positive. Clinical treatment and follow up were shown： case 1 was recurred 6 years postoperatively. Cases 2 and 3 had tumor removal and then radiation therapy. All three cases were followed up from 2. 4 years to 6 years, and currently were fine. Case 4 did right proximal femoral amputation and lost contact. Conclusions CCCS is a rare variant of low grade chondrosarcoma. The features of CCCS are different from conventional chondrosarcoma in terms of the clinical development, imaging, and pathology. In the practice,it should be differentiated from not only benign chondroblastoma and osteoblastoma, but also high grade clear cell osteosarcoma and metastatic clear cell carcinoma.

关 键 词：骨肿瘤 透明细胞软骨肉瘤 病理学 

分 类 号：R738.3[医药卫生—肿瘤]