Corradi Domenico, Bacchini Patrizia, Campanini Nicoletta, Bertoni Franco
Department of Pathology and Laboratory Medicine, Pathology Section, University of Parma, Via Gramsci 14, 43100 Parma, Italy.
Arch Pathol Lab Med. 2006 Nov;130(11):1673-9. doi: 10.5858/2006-130-1673-ACCCDD.
Clear cell chondrosarcoma (CCC) is commonly considered to be a low-grade subtype of chondrosarcoma. However, a few cases of CCC behave as high-grade lesions (with early metastases or multiple/synchronous locations).
To investigate morphologic features that can help predict the aggressiveness of these CCCs.
To investigate possible hallmarks of this aggressiveness, we are presenting the clinicopathologic features of 6 cases of CCC, 4 of which presented aggressive features and 2 low-grade behavior. The patients were 5 men and 1 woman; their ages ranged from 22 to 47 years. Histologic appearance, ultrastructure, and immunohistochemical expression of metalloproteinase 1 and 2 and their inhibitors were evaluated in all 6 cases.
Pain was the most common symptom; the lesions were located in the femur (4), humerus (2), and vertebral body (1), with 1 patient presenting a double/synchronous lesion. Although no major differences were detected using conventional light microscopy, an ultrastructural analysis--at variance with usual cases--showed a lack of superficial microvilli in more than 50% of neoplastic cells in the aggressive cases, therefore suggesting a less differentiated phenotype. In addition, metalloproteinase 2 was more diffusely expressed in the aggressive tumors than in the conventional CCCs, whereas p53 labeling was always negative.
The aggressive behavior of some CCCs may be, at least in part, correlated to a lesser degree of cell differentiation and to the expression of tumor cell proteins, such as metalloproteinase 2, which are able to favor neoplastic spreading.
透明细胞软骨肉瘤（CCC）通常被认为是软骨肉瘤的一种低级别亚型。然而，有少数CCC病例表现为高级别病变（伴有早期转移或多灶/同时性发病）。
研究有助于预测这些CCC侵袭性的形态学特征。
为了探究这种侵袭性的可能特征，我们呈现了6例CCC的临床病理特征，其中4例表现为侵袭性特征，2例表现为低级别行为。患者为5名男性和1名女性；年龄范围为22至47岁。对所有6例病例的组织学表现、超微结构以及金属蛋白酶1和2及其抑制剂的免疫组化表达进行了评估。
疼痛是最常见的症状；病变位于股骨（4例）、肱骨（2例）和椎体（1例），1例患者存在双发/同时性病变。虽然常规光学显微镜检查未发现重大差异，但超微结构分析——与通常病例不同——显示侵袭性病例中超过50%的肿瘤细胞缺乏表面微绒毛，因此提示为分化程度较低的表型。此外，金属蛋白酶2在侵袭性肿瘤中的表达比在传统CCC中更广泛，而p53标记始终为阴性。
部分CCC的侵袭性行为可能至少部分与细胞分化程度较低以及肿瘤细胞蛋白如金属蛋白酶2的表达有关，这些蛋白能够促进肿瘤扩散。
