The Journal of Bucharest College of Physicians and the Romanian Academy of Medical Sciences

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Sunday, November 18 2018 @ 04:51 EET

The Management of Facial Fibrous Dysplasia


V. Zainea, O. Ceachir, Mura Hainăroșie, Irina G Ioniță, Andreea Sorică, R. Hainăroșie

Fibrous dysplasia is a benign condition consisting in replacement of normal bone with fibrous tissue and unorganized bone woven (1). The malignant change to osteosarcoma can appear in less than 1% of cases (2). Osteosarcoma is found most often, but other lesions as fibrosarcoma, chondrosarcoma, and malignant fibrohistiocytoma are reported (3,4).

Fibrous dysplasia appears due to somatic activation of mutation in the G protein encoded by the gene GNAS. GNAS gene mutations cause McCune-Albright syndrome defined by the triad of PFD, café-au-lait skin macules and endocrinopathies, including amongothers, precocious puberty (5). It is caused by a random mutation in the GNAS gene that occurs very early in development.

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