摘要
目的探讨透明细胞软骨肉瘤(CCCS)的临床病理特征、诊断与鉴别诊断。方法结合临床资料及影像学所见,分析3例CCCS的组织病理学特征并复习相关文献。结果患者均为女性,年龄23～48岁,平均35.6岁。发病部位分别是鼻中隔、股骨及胫骨。X线显示溶骨性破坏,其内可见斑点状不规则钙化。病变界限清楚,具有薄层硬化边。巨检:灰白色肿瘤组织质软而脆。镜检:肿瘤呈弥漫片层状或模糊的分叶状排列,散在点缀着钙化或骨化灶。病变主要由温和的透明细胞构成,胞质丰富、空泛,边界清楚,核中位,无核沟,可见核仁;一些破骨细胞样巨细胞出现于条索状低级别软骨肉瘤及灶性反应性骨样组织中;其中2例继发动脉瘤样骨囊肿;所有病例核非典型及核分裂罕见。免疫组化:S-100和vimentin(+)。1例刮除术后病变复发并发生去分化。结论 CCCS是一种罕见的低度恶性软骨肉瘤的亚型,应与软骨母细胞瘤及转移性透明细胞癌等鉴别。确诊后推荐"大块切除"的外科手术治疗。
Objective To investigate the clinicopathologic features, diagnosis and differential diagnosis of clear cell chondrosarcoma （CCCS）. Methods Three cases of CCCS were analysed histopathologically in combination with clinical data and image findings, and the related literature was reviewed. Results All patients were females and their ages ranged from 23 to 53 years （mean,37.3）. Sites of involvement were the nasal septum, femur and tibia,respectively. X-ray revealed an osteolytic destruction which dotted with patchy or irregular calcification with a sharp demarcation and thin sclerotic rim. Grossly, the tumor was depicted as soft but gritty with a grey-white appearance. Microscopically, the lesion was arranged in a sheets or inconspicuous lobular pattern with scattered of calcified or ossified areas. The tumor consisted predominantly of bland clear cells with vaculated cytoplasm and distinct membranes. And, with large, centrally located nuclei, the clear cell had nucleolus but without groove. Among cords of conventional low grade chondrosarcoma components and foci of reactive osteoid tissues, some mild multinucleated giant cells were presented. And secondary aneurysmal bone cysts were also observed in 2 cases. However, nuclear atypia or mitotic figures were rare in all cases. Immunohistochemieally, the clear cells were positive for vimentin and S-100 protein. Among them, one case was local recurreneed with dedifferentiation after curettage. Conclusion CCCS is a rare, low-grade subtype of chondrosarcoma with special clinieopathological characteristics. It should be distinguished from some mimics such as chondroblastoma and metastatic clear cell carcinoma. And ＂en bloc＂ resection should be recommended when CCCS is identified.
出处
《诊断病理学杂志》
CSCD
北大核心
2013年第9期540-544,共5页
Chinese Journal of Diagnostic Pathology
关键词
骨肿瘤
透明细胞软骨肉瘤
临床病理
鉴别诊断
Bone neoplasms
Clear cell chondrosarcoma
Clinicopathology
Differential diagnosis
