Gaubert J, Durroux R
Service d'Orthopédie Pédiatrique de Toulouse, CHU Toulouse-Purpan.
Rev Chir Orthop Reparatrice Appar Mot. 1995;81(4):333-7.
Fibrous dysplasia and adamantinoma of the long bones are two different entities encountered on the same part of the tibial shaft. The relationship between these two diseases seems to be established. We describe a case with some common, features arguing for a single pathology.
A seven year old boy presented in 1974 with a destructive lesion involving the cortex of the tibial shaft. The patient subsequently presented several fractures, treated surgically and has been followed for nineteen and a half years. The first biopsy specimen in 1974 led to the diagnosis of ossifying fibroma or fibrous dysplasia. It was reviewed with immunohistochemical technics but no epithelial component could be found.
These pathologic aspects led to the diagnosis of adamantinoma associated with osteofibrous dysplasia of the tibia. Twenty five such cases were found in the literature. Two theories are proposed: 1) these two diseases are independent and coexist on the same bone; 2) there is only one disease with different features that are either those of fibrous dysplasia or those of adamantinoma. Immunohistochemical technics are needed for diagnosis.
Two sorts of adamantinoma can be observed on the long bones: adamantinoma without features of fibrous dysplasia with poor prognosis, adamantinoma with fibrous dysplasia's features called "differentiated adamantinoma" with a better prognosis.
骨纤维结构不良和长骨造釉细胞瘤是在胫骨干同一部位出现的两种不同疾病。这两种疾病之间的关系似乎已经确立。我们描述了一个具有一些共同特征的病例,支持单一病理学观点。
一名七岁男孩于1974年出现累及胫骨干皮质的破坏性病变。该患者随后出现多次骨折,接受了手术治疗,并已随访十九年半。1974年的首次活检标本诊断为骨化性纤维瘤或骨纤维结构不良。采用免疫组化技术对其进行复查,但未发现上皮成分。
这些病理表现导致诊断为与胫骨骨纤维发育异常相关的造釉细胞瘤。文献中发现了25例此类病例。提出了两种理论:1)这两种疾病是独立的,在同一骨上共存;2)只有一种具有不同特征的疾病,这些特征要么是骨纤维结构不良的特征,要么是造釉细胞瘤的特征。诊断需要免疫组化技术。
在长骨上可观察到两种类型的造釉细胞瘤:不具有骨纤维结构不良特征的造釉细胞瘤,预后较差;具有骨纤维结构不良特征的造釉细胞瘤,称为“分化型造釉细胞瘤”,预后较好。