Fibrous Dysplasia, Paget's Disease of Bone, and Other Uncommon Sclerotic Bone Lesions of the Craniofacial Bones

Abstract

Imaging studies of the brain, head and neck, sinuses, and dental computed tomography are among the most frequently performed procedures in radiologic departments. Systematic evaluation in the bone window may reveal common and uncommon sclerotic osseous abnormalities of the craniofacial skeleton.Most of these findings are incidental and unrelated to the initial clinical indications. Sporadically symptoms may arise due to lesional mass effect with compression on adjacent structures and neuroforaminal encroachment, resulting in proptosis, vision, or hearing loss. Other symptoms include craniofacial deformity, mandibular occlusion deformity, and local pain.This article reviews the most common disorders characterized by an increased bone density involving the craniofacial bones including fibrous dysplasia, Paget's disease of bone, meningioma with associated hyperostosis, and osteoma. Finally, typical examples of rarer sclerosing bone dysplasias are discussed as well.Emphasis is placed on imaging features and the differential diagnosis.

Coexistence of meningioma and craniofacial fibrous dysplasia: a case series of clinicopathological study and literature review

“…Both characterized by an increased bone density involving the craniofacial bones, meningioma associated hyperostosis and CFD can be confounded easily resulting in the dilemma to differentiate concomitant meningioma and CFD from meningioma with hyperostotic bone involvement. Seen in 25–49% of meningiomas [ 41 ], meningioma associated hyperostosis most frequently affects the convexity and sphenoid wing [ 5 , 42 ] and is featured by irregular inner surface margins and diffuse “hairy spicules” trabecular hyperostosis without the destruction of trabecular structures [ 43 – 45 ]. Additionally, as a special condition of meningioma restricted in bone (accounting for about 2%) [ 46 , 47 ], intraosseous meningiomas are readily evaluated as sclerotic lesions with irregular and spiculated borders [ 48 – 50 ].…”

“…Fibrous dysplasia (FD) is an uncommon mosaic disorder resulting in replacement of normal bone with fibro-osseous tissue. The actual incidence of FD is once reported to be 10–30 in 1,000,000 persons, representing as many as 7% of benign bone tumors [ 4 , 5 ]. It may occur in one single bone (monostotic FD), in multiple bones (polyostotic FD) or in combination with extra-skeletal disease.…”

“…Relevant differential diagnoses of CFD should consider meningiomas, Paget’s disease of the skull bone, and benign osteosclerotic lesions like osteoma [ 19 ]. Since meningioma itself could inflict the adjacent bones resulting in bone destruction with similar radiological manifestations to CFD [ 5 , 20 ], differential diagnosis between bone-invasive meningiomas and concomitant meningiomas and CFD is clinically problematic.…”

Frontal bone hyperostotic mass associated with fibrous dysplasia in a male patient with systemic lupus erythematosus (SLE)

“…Briefly speaking, hyperostosis is commonly defined as an excessive bone growth enabled in altering the normal subject's anatomy. A focal growth is usually found in disorders such as fibrous dysplasia (FD), meningiomas, ossifying fibroma, or hyperostosis frontalis interna (HFI) [2] , [3] . A case of hyperostosis in systemic lupus erythematosus (SLE) has been also reported quite recently [4] .…”