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Osteofibrous Dysplasia

Ossifying Fibroma, Juvenile Intracortical Adamantinoma, Differentiated Adamantinoma, Regressive Adamantinoma

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Bone and Soft Tissue Tumors

Abstract

These two lesions are so related as to deserve to be described under the same heading. Both are rare occurrences, but the adamantinoma is much more rare than osteofibrous dysplasia. One, osteofibrous dysplasia, is a benign even spontaneously regressing lesion, while the other, adamantinoma, is a low-grade malignant tumor even capable of metastasizing; there are, however, rare cases where an osteofibrous dysplasia changed into adamantinoma. Osteofibrous dysplasia is typical of small children, adamantinoma occurs in adults or adolescents; there are, however, exceptional cases of adamantinoma in children. Osteofibrous dysplasia is almost exclusive of the tibia (and fibula), adamantinoma is strikingly prevalent in the tibia, but can also occur in other long bones and even in the soft tissues. The clinico-imaging presentation of the two lesions can be different, but frequently it is quite similar or identical. Histologically, the osteofibrous dysplasia is characterized by a fibro-osseous pattern usually containing tiny foci of epithelial cells; adamantinoma is an epithelial tumor but frequently has an osteofibrous dysplasia-like tissue component.

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Campanacci, M. (1999). Osteofibrous Dysplasia. In: Bone and Soft Tissue Tumors. Springer, Vienna. https://doi.org/10.1007/978-3-7091-3846-5_44

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  • DOI: https://doi.org/10.1007/978-3-7091-3846-5_44

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