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Osteofibrous Dysplasia: Report of Two Cases with Literature Review

骨纖維發育不良症:兩病例報告與文獻回顧

摘要


骨纖維發育不良症是一種好發於十歲以下孩童的罕見骨纖維疾病。這種大多長在前脛骨的疾病有多樣的臨床表現,從無症狀到疼痛變形都有。影像上,骨纖維發育不良症呈現邊界清楚之骨皮質病灶。不論在影像學上或臨床病理學上,骨纖維發育不良症都和纖維性結構不良症及釉質上皮癌十分相似,也因此衍生了許多診斷及治療上的困擾。這篇文章除了介紹兩例年齡較不尋常的骨纖維發育不良症之外,也會將其臨床病理及影像學特徵和鑑別診斷作新的整理。

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並列摘要


Osteofibrous dysplasia (OFD) is a relatively uncommon osseous-fibrous disease that usually affects children under the age of ten. It has a strong predilection to anterior tibia with a various clinical presentation ranging from asymptomatic to painful bony deformity. Radiologically, OFD manifests as well-circumscribed intracortical lucency/lucencies. The clinicopathological and radiological similarities between OFD and fibrous dysplasia, classic adamantinoma and differentiated adamantinoma pose great challenges in terms of diagnosis and management of this often self-limiting disease. In this article, two cases of OFD that have a slightly unusual age of presentation will be presented. Current evidence regarding to the clinicopathologic and radio- logical features of OFD will also be summarized, along with discussion on its main differential diagnoses.

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