Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical, radiological, and histopathological features

Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensi...

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Published inJournal of oral pathology & medicine Vol. 48; no. 1; pp. 3 - 9
Main Authors Pereira, Thaís dos Santos Fontes, Gomes, Carolina Cavalieri, Brennan, Peter A, Fonseca, Felipe Paiva, Gomez, Ricardo Santiago
Format Journal Article
LanguageEnglish
Published Denmark Wiley Subscription Services, Inc 01.01.2019
Subjects
beta‐catenin
bone lesions
Dentistry
Differential diagnosis
Fibrous dysplasia
fibro‐osseous lesions
GNAS1
Pathogenesis
pathology
beta-catenin
fibro-osseous lesions
pathology
GNAS1
bone lesions
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Summary:Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune‐Albright syndrome, Jeffe‐Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management.
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ISSN:0904-2512
1600-0714
DOI:10.1111/jop.12797