长骨造釉细胞瘤临床病理分析及诊治方法

长骨造釉细胞瘤临床病理分析及诊治方法

摘要目的探讨长骨造釉细胞瘤(adamantinoma of long bone，ALB)的临床病理特征、鉴别诊断和治疗方法，以提高该病的诊断和治疗水平。方法分析8例ALB 的临床表现以及影像学、病理学特点，并复习相关文献。结果8例患者发病年龄为12～80岁，中位年龄33岁，女性3例，男性5例。影像学可表现为良性和（或）恶性肿瘤的特征。镜检：ALB的特征性改变主要由上皮细胞和梭形细胞间质共同组成，两者以不同的比例和形态相互交织排列。鳞状细胞型5例、管状型1例、基底细胞型1例、纤维结构不良型1例。免疫组化：上皮细胞表达CK、EMA，部分表达vimentin，不表达CD34，纤维成分表达vimentin。6例患者采用扩大切除加局部重建术，术后随访1～12年，2例失访、1例术后感染愈合不佳外，其余3例恢复良好。剩余2例患者采用肿瘤刮除术，术后1例患者20天死亡，1例患者术后2年复发。结论ALB是以形态多样为特征的低级别双向分化的恶性肿瘤，影像学鉴别诊断比较困难，需结合肿瘤的组织学形态和免疫组化特点共同诊断。局灶刮除术易复发、转移或死亡，应采用扩大切除术，并长期密切随访。

关键词：长骨肿瘤；造釉细胞瘤；病理特征；鉴别诊断；治疗方法Clinicopathological features, diagnosis and treatment of adamantinoma of long bone Abstract Object To explore the clinicopathological features, differential diagnosis and treatment of adamantinoma of long bone(ALB), in order to improve the diagnosis and treatment of the disease. Method The clinical manifestations, imaging and pathological features of 8 cases of ALB were analyzed, and relevant literatures were reviewed. Results The age of the 8 patients ranged from 12 to 80 years(median 33). Among the 8 cases , 3 cases were females and 5 males. Imaging can be characterized by benign and/or malignant tumors. The epithelial and osteofibrous components belonged to the characterizations of ALB, which were intermingled with each other in various proportions and differentiation patterns. Squamous cell type 5 cases, tubular type 1 case, basal cell type 1 case, fibrous dysplasia type 1 case. The epithelial cells were expressed CK and EMA, fibrou stroma partial epithelial cells were vimentin positive, while CD34 in epithelial cells were negative. 6 patients underwent extended resection and local reconstruction. Follow-up for 1 to 12 years

showed that 2 patients lost follow-up, 1 patient had poor healing of postoperative infection, and the other 3 patients recovered well. 1 patient died 20 days after underwent tumor curettage and another case of ALB was found recurrece follow-up 2 years. Conclusion ALB, characterized by a variety of morphological patterns, is a low grade malignant biphasic tumor. Imaging differential diagnosis is difficult. The diagnosis and differential diagnosis of adamantinoma shuold be made by combination of histopathological alternations with immunohistochemical findings. Focal curettage is prone to relapse, metastasis or death. Extended resection should be adopted and long-term close follow-up should be conducted.

Keywords: Long bone tumor; Adamantinoma ; Pathological features; Differential diagnosis; Treatment method

长骨造釉细胞瘤(adamantinoma of long bone，ALB)是一种罕见的局部侵袭性较强的低度恶性骨原发肿瘤，在骨肿瘤中占0.4%，且80～90%好发于胫骨中段[1]。因ALB罕见且临床症状不典型，影像学上可表现出骨质破坏，病理学上上皮成分似异型的鳞状细胞在纤维性成分中浸润性生长，故临床上常与转移癌、骨纤维结构不良、骨巨细胞瘤、纤维肉瘤、造釉细胞瘤样尤文肉瘤等骨良恶性病变难以鉴别。我们收集8例ALB，结合文献分析其临床病理特点、影像学表现、鉴别诊断，并探讨其治疗方法与预后。

1材料与方法

1.1材料收集南京市鼓楼医院病理科于2003年5月至2019年5月诊断的ALB 7例（2例为会诊病例）和徐州医科大学附属连云港市第一人民医院病理科于2008年3月至2019年3月诊断的ALB 1例患者的临床和病理资料进行回顾分析。1.2方法8例送检标本均经10%中性福尔马林固定，石蜡包埋，4 m厚连续切片，HE染色，光镜观察。免疫组化采用EnVision两步法，DAB显色，以PBS 代替一抗作为空白对照，免疫组化所用抗体CK、EMA、CD34、p63、S-100、vimentin、P40、CK7、CK20、Ki-67均购自广州安必平生物科技有限公司。

2 结果

2.1 临床资料本组8例患者女性3例，男性5例，发病年龄为12～80岁，中位年龄33岁，发病部位胫骨7例（6例为中段，1例中下段），股骨近端1例。8例患者