长骨造釉细胞瘤临床病理分析及诊治办法摘要 目的 探讨长骨造釉细胞瘤(adamantinoma of long bone，ALB)的临床病理特性、鉴别诊疗和治疗办法，以提高该病的诊疗和治疗水平。办法 分析8例ALB的临床体现以及影像学、病理学特点，并复习有关文献。成果 8例患者发病年纪为12～80岁，中位年纪33岁，女性3例，男性5例。影像学可体现为良性和（或）恶性肿瘤的特性。镜检：ALB的特性性变化重要由上皮细胞和梭形细胞间质共同构成，两者以不同的比例和形态互相交错排列。鳞状细胞型5例、管状型1例、基底细胞型1例、纤维构造不良型1例。免疫组化：上皮细胞体现CK、EMA，部分体现vimentin，不体现CD34，纤维成分体现vimentin。6例患者采用扩大切除加局部重建术，术后随访1～，2例失访、1例术后感染愈合不佳外，其它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是以形态多样为特性的低档别双向分化的恶性肿瘤，影像学鉴别诊疗比较困难，需结合肿瘤的组织学形态和免疫组化特点共同诊疗。局灶刮除术易复发、转移或死亡，应采用扩大切除术，并长久亲密随访。核心词：长骨肿瘤；造釉细胞瘤；病理特性；鉴别诊疗；治疗办法Clinicopathological features, diagnosis and treatment of adamantinoma of long boneAbstract 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 dysplasi...
