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Osteofibrous Dysplasia with Rhabdoid Elements in a 38-Year-Old Man with Spontaneous Regression Over Five Years

A Case Report

Exner, G. Ulrich, MD1,a; von Hochstetter, Arthur R., MD2; Pfirrmann, Christian W.A., MD3

doi: 10.2106/JBJS.CC.17.00294
Case Reports

Case: A 38-year-old man presented with multifocal, partially confluent osteolytic lesions in the proximal dia-meta-epiphyseal region of the proximal aspect of the left tibia, which had been found incidentally when a radiograph was made after a rotational knee injury. When the results of a percutaneous core needle biopsy proved inconclusive, an open biopsy was performed. Osteofibrous dysplasia (OFD) with scattered groups of plump cells with a rhabdoid phenotype, shown to express both vimentin and pan-cytokeratin, was found. Because the lesion was an incidental finding, we decided to proceed with observation. Three months after the open biopsy, imaging showed marked regression of the lesion; there was nearly complete normalization 5 years later.

Conclusion: To our knowledge, there has been only 1 prior reported case with these pathologic features, and there have been no reports of complete spontaneous regression in an adult patient with OFD. Treatment recommendations for OFD and for OFD-like adamantinoma range from observation to aggressive resection.

1Orthopaedie Zentrum Zurich, Zurich, Switzerland

2Pathologie Institut Enge, Zurich, Switzerland

3Department of Radiology, Universitaetsklinik Balgrist, Zurich, Switzerland

aE-mail address for G.U. Exner:

Copyright © 2018 by The Journal of Bone and Joint Surgery, Incorporated
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