Zheng Li, Zhang Hui-zhen, Huang Jin, Tang Juan, Liu Liang, Jiang Zhi-ming
Department of Pathology, Shanghai 6th People's Hospital, Shanghai Jiaotong University, Shanghai 200233, China.
Zhonghua Bing Li Xue Za Zhi. 2009 Sep;38(9):609-13.
To investigate the clinicopathologic, radiological and immunohistochemical characteristics of osteochondroma with malignant transformation.
The clinical data, radiological imagings and hematoxylineosin stained histologic sections were reviewed in 463 cases of osteochondroma diagnosed in Shanghai 6th Hospital from 1991 to 2008, including 11 cases with malignant transformation. Immunohistochemical two-step staining was used to detect CK, vimentin, S-100 protein, p53 and c-myc expression in seven cases of osteochondroma with malignant transformation and 10 cases without malignant transformation. The relevant literature was reviewed.
Among the 11 cases with malignant transformation, five were single osteochondroma (5/408, 1.2%), and six were multiple osteochondromas (6/55, 10.9%). The male to female ratio was 10:1. These 11 cases were derived from femur (4 cases), tibia (3 cases), ilium (3 cases), shoulder bone (1 case) and pubis (1 case). There was one case that showed malignant transformation in both the femur and ilium. The mean ages for the malignant and non-malignant cases were 39.8 years and 20.4 years respectively. All the malignant cases showed large sized lesions with prominent calcification in the thick cartilage caps. The malignant component was low grade, peripheral chondrosarcoma (grade I-II). In some areas the tumor cells infiltrated the peripheral soft tissue and bone marrow. Of the seven cases with malignant transformation that had immunohistochemical staining, all were positive for vimentin and S-100 protein; p53 protein was positive in 2 of 7 cases.
Malignant transformation of osteochondroma was usually encountered in multiple lesions. Most patients were more than 30 years old with a long clinical history and with a male predominance. These tumors showed thick cartilage caps with prominent calcification. The lobulated nature of the tumors was evident and they infiltrated the surrounding soft tissue. The sarcomatoid component was peripheral type, well differentiated chondrosarcoma. p53 mutation may explain part of the molecular mechanism in the malignant transformation.
探讨骨软骨瘤恶变的临床病理、影像学及免疫组化特征。
回顾性分析1991年至2008年在上海第六人民医院诊断的463例骨软骨瘤患者的临床资料、影像学表现及苏木精-伊红染色组织切片，其中11例发生恶变。采用免疫组化两步法检测7例恶变骨软骨瘤及10例未恶变骨软骨瘤中细胞角蛋白（CK）、波形蛋白、S-100蛋白、p53和c-myc的表达情况，并复习相关文献。
11例恶变患者中，单发性骨软骨瘤5例（5/408，1.2%），多发性骨软骨瘤6例（6/55，10.9%）。男女比例为10∶1。这11例分别来源于股骨（4例）﹑胫骨（3例）﹑髂骨（3例）﹑肩胛骨（1例）和耻骨（1例），其中1例股骨和髂骨均发生恶变。恶变组和未恶变组的平均年龄分别为39.8岁和20.4岁。所有恶变病例均表现为病变较大，厚软骨帽内有明显钙化。恶性成分是低级别周围型软骨肉瘤（Ⅰ-Ⅱ级）。部分区域肿瘤细胞浸润周围软组织和骨髓。7例进行免疫组化染色的恶变病例中，波形蛋白和S-100蛋白均呈阳性；7例中有2例p53蛋白呈阳性。
骨软骨瘤恶变多见于多发病变。多数患者年龄超过30岁，临床病史长，男性居多。这些肿瘤表现为厚软骨帽伴明显钙化。肿瘤呈分叶状，明显浸润周围软组织。肉瘤样成分是周围型，为高分化软骨肉瘤。p53突变可能是恶变分子机制的一部分。
