16: Nonodontogenic Intraosseous Lesions

16 Nonodontogenic Intraosseous Lesions

Tori and Exostoses

Fibro-Osseous Lesions

Fibro-osseous lesions are a heterogeneous group of lesions composed of osseous dysplasias, inflammatory conditions, and neoplasms, characterized by a proliferation of fibroblast-like cells and deposition of osseous or cementum-like material. Their histologic features overlap, depending on the stage in which the biopsies are obtained, and an accurate diagnosis can be rendered only after correlation of clinical and radiographic data. Differentiation between lesions is essential so that appropriate therapy can be instituted. The lesions considered here are fibrous dysplasia, cemento-osseous dysplasia, cemento-ossifying fibroma, aggressive ossifying fibroma, and familial gigantiform cementoma. Nomenclature for these lesions continues to evolve.

Clinical Findings


FIGURE 16-5 Fibrous dysplasia. A, Periapical films showing ground-glass appearance of the right maxilla (top panel) and normal trabeculation on the left (bottom panel). B, Ground-glass appearance of mandibular bone.

(A, Courtesy of Dr. Bernard Friedland, Harvard School of Dental Medicine, Boston, Mass; B, Courtesy of Dr. Stanley Hirsch, Case Western Reserve University, Cleveland, Ohio.)


FIGURE 16-9 Aggressive ossifying fibroma: huge radiolucent and radiopaque lesion of the maxilla.

(Courtesy of Dr. Bonnie Padwa, Harvard School of Dental Medicine, Boston, Mass.)


FIGURE 16-10 Familial gigantiform cementomas: marked expansion of maxilla (A) and mandible (B) by radiolucent-radiopaque process.

(From Abdelsayed RA, Eversole LR, Singh BS, Scarbrough FE. Gigantiform cementoma: clinicopathologic presentation of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;91:438-444.)

Etiopathogenesis and Histopathologic Features

Fibrous dysplasia is associated with a mutation in the GNAS gene that results in alterations in proliferation and differentiation of preosteoblasts; there are monostotic (85% to 90% of cases) and polyostotic types; the latter is seen in McCune-Albright syndrome and is often associated with endocrinopathies and skin pigmentation. The other conditions have not been shown to be consistently associated with gene mutations, although familial gigantiform cementoma is an inherited disorder.

Fibrous dysplasia consists of a proliferation of benign fibrous tissue and either small, delicate, or curvilinear trabeculae of woven bone in early lesions, or thicker and denser trabeculae of woven or lamellar bone in mature lesions; osteoblastic rimming and occasional osteoclasts may be seen focally, especially in early and inflamed lesions; there is variable vascularity (Figs. 16-11 and 16-12); aneurysmal bone cyst–like areas may be seen.

Cemento-osseous dysplasia consists of a cellular proliferation of spindled benign fibroblast-like cells and deposition of osteoid, woven bone, cementum droplets, or a mixture thereof; mature lesions consist of a mass of cementum that has a rounded, globular appearance with few cementocytes (sclerotic cemental mass) (Figs. 16-13 to 16-15); cells that rim bone and cementum are often osteoblast-like.

Cemento-ossifying fibroma consists of cellular proliferation of spindled fibroblast-like cells that deposit osteoid, trabeculae of woven bone, and cementum droplets in varying proportions; osteoblastic rimming is common (Fig. 16-16).

Aggressive ossifying fibroma (desmo-osteoblastoma) consists of a cellular proliferation of plump and stellate fibroblast-like cells with large, slightly atypical nuclei, clusters of multinucleated giant cells, and fresh hemorrhage; trabecular variant has abundant woven bone surrounding large osteocytes with many osteoblasts (Figs. 16-17 and 16-18); psammommatoid variant has many basophilic cementum droplets or psammomatoid bodies, most of which are of uniform size (Fig. 16-19); this subtype appears similar to familial gigantiform cementoma.

Familial gigantiform cementoma is similar to ossifying fibroma, especially psammomatoid type.


Abdelsayed RA, Eversole LR, Singh BS, Scarbrough FE. Gigantiform cementoma: clinicopathologic presentation of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;91:438-444.

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Eversole LR, Leider AS, Nelson K. Ossifying fibroma: a clinicopathologic study of sixty-four cases. Oral Surg Oral Med Oral Pathol. 1985;60:505-511.

Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck Pathol. 2008;2:177-202.

MacDonald-Jankowski D. Fibrous dysplasia: a systematic review. Dentomaxillofac Radiol. 2009;38:196-215.

Macdonald-Jankowski DS, Li TK. Fibrous dysplasia in a Hong Kong community: the clinical and radiological features and outcomes of treatment. Dentomaxillofac Radiol. 2009;38:63-72.

Rossbach HC, Letson D, Lacson A, et al. Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: a possible explanation of an ancient mystery. Pediatr Blood Cancer. 2005;44:390-396.

Su L, Weathers DR, Waldron CA. Distinguishing features of focal cemento-osseous dysplasias and cemento-ossifying fibromas. I. A pathologic spectrum of 316 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997;84:301-309.

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Summerlin DJ, Tomich CE. Focal cemento-osseous dysplasia: a clinicopathologic study of 221 cases. Oral Surg Oral Med Oral Pathol. 1994;78:611-620.

Young SK, Markowitz NR, Sullivan S, et al. Familial gigantiform cementoma: classification and presentation of a large pedigree. Oral Surg Oral Med Oral Pathol. 1989;68:740-747.

Paget Disease of Bone (Osteitis Deformans)

Paget disease of bone is a metabolic bone disease that leads to disorganized deposition of bone that is dense but has poor mechanical strength and, therefore, is prone to fractures.

Etiopathogenesis and Histopathologic Findings

Both genetic and environmental influences have been identified. Some cases are inherited in an autosomal dominant fashion; sequestosome, a gene that encodes p62 (a protein involved in NFκB signaling) is present in 20% to 50% of inherited and 5% to 20% of sporadic cases; increased number and activity of osteoclasts leads to initial resorption of bone and osteoporotic appearance (osteoclastic stage), followed by compensatory but overproduction of lamellar bone in a haphazard fashion with prominent vascularity (mixed osteoclastic-osteoblastic stage), and finally, “burnt out” stage with fibrosis of connective tissue; the role of paramyxovirus and other viruses (such as measles and distemper virus) as a trigger is controversial.

Central Giant Cell Granuloma (Aggressive and Nonaggressive Giant Cell Lesion, Central Giant Cell Lesion, Central Giant Cell Reparative Granuloma)

Data suggest that the nonaggressive and aggressive giant cell granulomas and lesions of the maxillofacial complex have similar clinical presentations and treatment outcomes when compared, respectively, with the nonaggressive and aggressive axial-appendicular giant cell lesions; therefore, they may represent the same disease process.

Etiopathogenesis and Histopathologic Features

Cells are derived from mesenchymal stromal cells that differentiate along the monocyte-macrophage-osteoclast lineage. No consistent genetic abnormalities have been noted, although frequent telomeric associations have been reported in giant cell tumor of bone.


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de Lange J, van Rijn RR, van den Burg H, et al. Regression of central giant cell granuloma by a combination of interferon and imatinib: a case report. Br J Oral Maxillofac Surg. 2009;47:59-61.

Dewsnup NC, Susarla SM, Abulikemu M, et al. Immunohistochemical evaluation of giant cell tumors of the jaws using CD34 density analysis. J Oral Maxillofac Surg. 2008;66:928-933.

Gebre-Medhin S, Broberg K, Jonson T, et al. Telomeric associations correlate with telomere length reduction and clonal chromosome aberrations in giant cell tumor of bone. Cytogenet Genome Res. 2009;124:121-127.

Gleason BC, Kleinman PK, Debelenko LV, et al. Novel karyotypes in giant cell-rich lesions of bone. Am J Surg Pathol. 2007;31:926-932.

Kaban LB, Troulis MJ, Wilkinson MS, et al. Adjuvant antiangiogenic therapy for giant cell tumors of the jaws. J Oral Maxillofac Surg. 2007;65:2018-2024. discussion 2024

Liu B, Yu SF, Li TJ. Multinucleated giant cells in various forms of giant cell containing lesions of the jaws express features of osteoclasts. J Oral Pathol Med. 2003;32:367-375.

Resnick CM, Margolis J, Susarla SM, et al. Maxillofacial and axial/appendicular giant cell lesions: unique tumors or variants of the same disease? A comparison of phenotypic, clinical, and radiographic characteristics. J Oral Maxillofac Surg. 2010;68:130-137.

Tobon-Arroyave SI, Franco-Gonzalez LM, Isaza-Guzman DM, et al. Immunohistochemical expression of RANK, GRalpha and CTR in central giant cell granuloma of the jaws. Oral Oncol. 2005;41:480-488.

Vered M, Buchner A, Dayan D. Immunohistochemical expression of glucocorticoid and calcitonin receptors as a tool for selecting therapeutic approach in central giant cell granuloma of the jawbones. Int J Oral Maxillofac Surg. 2006;35:756-760.

Whitaker SB, Waldron CA. Central giant cell lesions of the jaws. A clinical, radiologic and histopathologic study. Oral Surg Oral Med Oral Pathol. 1993;75:199-208.

Jan 12, 2015 | Posted by in Oral and Maxillofacial Pathology | Comments Off on 16: Nonodontogenic Intraosseous Lesions
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