ABSTRACT

‘Fibrous dysplasia of the bone, a pathologic entity of the bone characterized by extensive proliferation of fibrous tissue, which destroys and replaces normal bone elements, was differentiated from generalized fibrocystic disease of the bone caused by hyperfunction of the parathyroid glands by Hunter, et al’ 1 2 in 1931. The classic clinical, pathologic and roentgenographic appearance was described by Louis Lichtenstein 5 (Hospital for Joint Disease, New York) in 1938. He reported eight cases of polyostotic fibrous dysplasia drawn to his attention by Henry Jaffe. The first case report in the article concerns a 19-year-old woman whose right femur roentgenograms showed ‘broadening and rarefaction of the upper two thirds. The cortex of the bone was thin … the osseous structure had a hazy, ground glass, trabeculated appearance’ 3 (Figure 1). A follow-up article by Lichtenstein and Jaffe 4 further explained the radiographic findings by stating, ‘The rarefactions reflect the replacement, in the affected area, of the spongy bone and of the adjacent inner surface of the cortex by fibrous connective tissue, which, of course, is relatively radiolucent. If within this tissue there has been substantial metaplastic ossification, the rarefaction shadow is likely to present a mottled or rather cloudy ground glass appearance.’ 4 Recognition of a bone tumor’s matrix mineralization pattern (fibrous, chondroid or osseous) is one of the key factors involved in making a specific diagnosis. The ground glass pattern is indicative of a fibrous tissue matrix (Figures 2 and 3). ‘Roentgenogram of a portion of a diseased tibia [on right], illustrating the marked thinning of the cortex, the absence of deposition of periosteal bone, the endosteal erosion of the cortex and the filling of the medullary cavity by a homogeneous fibrous tissue containing tiny spicules of bone. Compare with the normal tibia on the left.’ Reprinted from Lichtenstein<xref ref-type="bibr" rid="ref36_3"> <sup>3</sup> </xref>. Polyostotic fibrous dysplasia. <italic>Arch. Surg.,</italic> 1938, 36, 874–98. Copyright 1938, American Medical Association https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9781003076568/34c29fd5-5662-4e8f-b58c-badd7fa5d724/content/fig36_1_B.jpg"/> Typical fibrous dysplasia of the tibia (author’s case) https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9781003076568/34c29fd5-5662-4e8f-b58c-badd7fa5d724/content/fig36_2_B.jpg"/> Photograph (A) and radiograph (B) of tibia. Medullary space filled with ground glass. Ground glass courtesy of Sylvio and Jean Bettio, Chipped Glass and Crystal Repair Service, Clifton, NJ. Specimen radiograph by Jay Meyers, Registered Radiologic Technologist (RRT) https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9781003076568/34c29fd5-5662-4e8f-b58c-badd7fa5d724/content/fig36_3_B.jpg"/>