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Fibrous Dysplasia Of The Maxilla And Mandible- Our 5-year Institutional Experience In Light Of Literature Review | 110403
ISSN: 2161-0681
Journal of Clinical & Experimental Pathology
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Fibrous dysplasia is a benign intraosseous lesion. It is not a neoplasm, but a developmental condition of the bone
which appears similar to a tumor on radiological studies. Fibrous dysplasia is a sporadic condition caused by
the inability to produce mature bone due to a genetic mutation in GNAS1; it can present in two clinical forms,
monostotic or polyostotic. Fibrous dysplasia (FD) can be a component of McCune-Albright syndrome with both
dermatological and endocrinological features. The femur, ribs and maxillofacial skeleton are the most common
sites of involvement. Monostotic forms account for 80-85 % of the cases. It is caused by somatic activating
mutations in the GNAS gene, which lead to constitutive activation of adenylyl cyclase and elevated levels of cyclic
AMP.
Herein, we report of six cases of fibrous dysplasia of the mandible and maxilla in our facility between November
2015 and October 2020 with mean age 44 years (range, 12-72 years). The maxilla was affected in the most cases
(n=4), compared to mandible (n=2). The male to female ratio was 2:1. Some of the lesions showed ground-glass
opacity on imaging. Microscopic examination of the biopsied specimens from each patient revealed fragments
of relatively dense fibrous tissue containing irregularly shaped trabeculae of woven and lamellar bone. Some
trabeculae were associated with newly forming, spontaneous bone derived from the surrounding fibrous tissue
consistent with fibrous dysplasia. Each case demonstrated blending of fibrous and osseous tissue, with resultant
secondary bony metaplasia, producing immature, haphazard, and weakly calcified woven bone.
The clinical, radiographic, and the morphological appearance of FD exhibit a substantial overlap with other fibroosseous
lesions, including malignant neoplasms, such as low grade osteosarcoma. FD involves the maxilla almost
two times more often than the mandible. It frequently appears in the posterior region of the jaw bone and is
usually unilateral. The importance of this study is to differentiate FD from other fibro-osseous lesions of the
mandible and maxilla.
Biography
Dr. Daniela Proca is Professor of Clinical Pathology at Temple University, Philadelphia, Pennsylvania, USA and studies together with colleagues and residents bone and soft tissue lesions, particularly neoplasms.
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