Ueda Y, Roessner A, Bosse A, Edel G, Böcker W, Wuisman P
Gerhard-Domagk-Institute for Pathology, University of Münster, FRG.
Pathol Res Pract. 1991 Dec;187(8):1039-43; discussion 1043-4. doi: 10.1016/s0344-0338(11)81081-2.
In view of the still disputed relationship between adult adamantinoma and osteofibrous dysplasia in children, a unique case of adamantinoma, indicating a direct relationship between the two lesions, is presented with a review of the literature. The patient was a six-year-old boy who complained of pain and swelling in the left lower leg. Roentgenographs showed a loculate osteolysis surrounded by sclerosis within the cortex of the tibial shaft that would be typical of osteofibrous dysplasia. Although an osteofibrous-dysplastic component predominated histologically, some small islands of epithelial cells were scattered throughout the lesion. Immunohistochemically, the tumor cells of these epithelial islands gave a constant positive reaction for cytokeratin as well as vimentin, while the stromal cells in the osteofibrous dysplasia-like lesion were positive for vimentin only. This type of lesion is recorded in the Bone Tumor Registry of Westphalia at a rate of 8.3% for osteofibrous dysplasia, and of 25% for adamantinoma. A review of the literature, yielding reports with remarkable uniformity on 14 cases beyond the present one, suggests the existence of a separate clinicopathologic entity to be called juvenile intracortical adamantinoma with predominant osteofibrous dysplasia-like features, and which might be a regressing form of adamantinoma specific in childhood.
鉴于成人造釉细胞瘤与儿童骨纤维结构不良之间的关系仍存在争议，本文报告了一例独特的造釉细胞瘤病例，该病例表明这两种病变之间存在直接关系，并对相关文献进行了综述。患者为一名6岁男孩，主诉左小腿疼痛和肿胀。X线片显示胫骨干皮质内有一个被硬化带包围的分叶状骨质溶解区，这是骨纤维结构不良的典型表现。尽管组织学上以骨纤维结构不良成分占主导，但整个病变中散在分布着一些小的上皮细胞岛。免疫组化显示，这些上皮岛的肿瘤细胞对细胞角蛋白和波形蛋白均呈持续阳性反应，而骨纤维结构不良样病变中的基质细胞仅对波形蛋白呈阳性反应。在威斯特伐利亚骨肿瘤登记处，这种类型的病变在骨纤维结构不良中的记录发生率为8.3%，在造釉细胞瘤中的记录发生率为25%。对文献的综述显示，除本文病例外，另有14例报告具有显著的一致性，提示存在一种单独的临床病理实体，可称为具有主要骨纤维结构不良样特征的青少年皮质内造釉细胞瘤，它可能是儿童期特有的造釉细胞瘤的一种退行性形式。
