Ueda Y, Blasius S, Edel G, Wuisman P, Böcker W, Roessner A
First Department of Surgery, Kyoto University, Faculty of Medicine, Japan.
J Cancer Res Clin Oncol. 1992;118(2):152-6. doi: 10.1007/BF01187505.
The most controversial aspect of osteofibrous dysplasia (OFD) is its possible histogenetic relationship to adamantinoma of long bone. Evidence is recently beginning to accumulate that OFD may be a reactive process to regressive adamantinoma. To verify the concept, 13 lesions of OFD were studied again by immunohistochemistry for cytokeratins of different molecular masses, as well as by conventional stainings. In addition, 2 adamantinomas and 6 fibrous dysplasias of the tibia were studied for reference. A small number of spindle- or ovoid-shaped cells scattered individually in the fibro-osseous stroma showed positive reactions for cytokeratins of 55-57 kDa in 2 lesions, and for those of 45-56.5 kDa in 8 lesions of 13 OFDs, although no definite epithelial island could be detected even by immunohistochemistry. Adamantinomas also showed single cytokeratin-positive cells dispersed in fibroblastic stroma, in addition to epithelial islands positive for cytokeratins of both 55-57 kDa and 45-56.5 kDa. All cases of fibrous dysplasia were negative for cytokeratins. During the observation, no case of OFDs progressed to classic adamantinoma. The present study, demonstrating the existence of an intermediate stage between "differentiated adamantinoma" and total elimination of adamantinomatous components, gives further support for the concept that OFD is a secondary reactive process to adamantinomatous tissue. In practice, the existence of single scattered cytokeratin-immunoreactive cells in otherwise typical OFDs may not indicate the truly malignant behaviour of classic adamantinoma, unless discrete epithelioid cell nests are also found.
骨纤维结构不良（OFD）最具争议的方面是其与长骨造釉细胞瘤可能的组织发生关系。最近有证据开始积累，表明OFD可能是对退行性造釉细胞瘤的一种反应性过程。为了验证这一概念，对13例OFD病变再次进行了不同分子质量细胞角蛋白的免疫组织化学研究以及传统染色。此外，还研究了2例造釉细胞瘤和6例胫骨骨纤维发育异常作为对照。在13例OFD中，有2例在纤维性骨基质中单个散在分布的少数梭形或卵圆形细胞对55 - 57 kDa的细胞角蛋白呈阳性反应，8例对45 - 56.5 kDa的细胞角蛋白呈阳性反应，尽管即使通过免疫组织化学也未检测到明确的上皮岛。造釉细胞瘤除了有对55 - 57 kDa和45 - 56.5 kDa细胞角蛋白均呈阳性的上皮岛外，还显示单个细胞角蛋白阳性细胞散在于成纤维细胞基质中。所有骨纤维发育异常病例的细胞角蛋白均为阴性。在观察期间，没有OFD病例进展为典型造釉细胞瘤。本研究证明了在“分化型造釉细胞瘤”和造釉细胞瘤成分完全消失之间存在一个中间阶段，进一步支持了OFD是对造釉细胞瘤组织的继发性反应性过程这一概念。在实际应用中，在其他方面典型的OFD中单个散在的细胞角蛋白免疫反应阳性细胞的存在可能并不表明典型造釉细胞瘤具有真正的恶性行为，除非还发现离散的上皮样细胞巢。
