Fibrous dysplasia of bone and concomitant dysplastic changes in the dentin

Dental perspectives in fibrous dysplasia and McCune–Albright syndrome

“…FD lesions can grow rapidly, leading to bone expansion and displacement of adjacent structures such as the orbit and teeth 13, 20, 27, 28 . The metabolic dysfunctions and disordered bone architecture in FD/MAS can potentially affect tooth development and eruption 29, 30 . Although FD is a disease of the skeletal stem cell/osteoblastic lineage in which excess cAMP impairs the ability of the stem cell to differentiate to mature functioning osteoblasts 9 , it is unclear whether excess cAMP affects the developing tooth, either directly or indirectly.…”

“…Also, some dental healthcare providers may also feel uneasy about treating FD/MAS patients because of previous subjective reports that dental surgery might exacerbate jaw FD, transforming a quiescent lesion to an aggressively growing lesion 32, 33, 40 . There are also concerns regarding bisphosphonate-associated osteonecrosis of the jaw (ONJ) 29, 30, 32, 34, 35, 37 . However, ONJ is rare in FD/MAS, possibly due to the relatively lower bisphosphonate dosing regimens compared to doses used in cancer patients, or perhaps due to the relatively hypervascular nature of FD.…”

Albright's Syndrome: Review of the Literature and Case Report.

“…This is a variation of Albright's syndrome, but is not as serious . Albright's syndrome is characterized by the triangle of polyostotic fibrous dysplasia, "cafe au lait" spots on the skin and endocrinopathies of unknown etiology and uncertain pathogenesis [3][4][5][6][7][8][9][10]. Females are affected more often in a proportion of 3:1; monostotic fibrous dysplasia is 20 to 30 times more frequent than the polyostotic form.…”

“…Initially these lesions appear as radiolucent spots and can also acquire a radiopaque aspect; approximately 15% of all cases of polyostotic dysplasia involve the maxilla or mandible, the most common clinical sign being facial asymmetry without painful symptoms. Occasionally there are reports of dentinal dysplasia in areas of fibrous dysplasia [9] and delay of tooth eruption. In some cases dentinal resorption is noted [4,16].…”

“…This aspect depends on the age of the lesion [2,3,9]. Eventually, the presence of cartilaginous tissue can be observed within the connective tissue due to osseous metaplasia [3,17,18].…”