Abstract
Lesions denominated fibro-osseous lesions of the jaw constitute a diversified group of disorders, in which the normal bone architecture is replaced by fibroblasts, collagen fibers and immature bone. At present, the World Health Organization recognizes four variants of these lesions, namely: bone-cement dysplasia, fibrous dysplasia, ossifying fibroma and Familial gigantiform cementoma. Fibrous dysplasia may present in the monostotic form, affecting one single bone or an isolated craniofacial region; and in the Polyostotic form, involving two or more bones of the skeleton, and eventual association with syndromic conditions. The patient, C.P.G., 43 years old, sought attendance due to symptomatic increase in the region of the mandibular body on the right side. Imaging exams revealed craniofacial areas with ground-glass aspect, beyond the extensive mandibular radiolucent lesion. During the physical exam, spots of the Café au lait type disposed on the right side of the body were identified, in addition to uncoordinated gait with distinct shortening of the right leg. Additional radiographic exams showed evidence of skeletal dissemination of the disease. The patient denied any sexual precocity, and the final diagnosis was fibrous dysplasia, expressed by means of the Jaffe-Lichtenstein syndrome, in association with a simple bone cyst.
Highlights
The term fibro-osseous used with reference to a diversified group of disorders, in which the normal bone architecture is replaced by fibroblasts, collagen fibers and immature bone [1]
The simple bone cyst or idiopathic bone cavity has been related to fibro-osseous lesions, among them, florid cemento-osseous dysplasia is especially outstanding [3,4]
Fibrous dysplasia is a disease characterized as a benign bone developmental disorder, with slow progression, frequently associated with mutation of the GNAS1 gene and with stabilization after puberty [5,9]
Summary
The term fibro-osseous used with reference to a diversified group of disorders, in which the normal bone architecture is replaced by fibroblasts, collagen fibers and immature bone [1]. In these lesions a connective tissue matrix is identified, containing variable quantities of mineralized tissue that may be medullary or cementoid bone [1]. Despite the uncertain etiology of fibrous dysplasia, the involvement of activating mutation in the GNAS1 gene has been widely described This in turn encodes the Gs alpha protein in pluripotent embryonic stem cells, predisposing them to skeletal changes [5,6]. The clinical, imaging, laboratory, histopathological and therapeutic characteristics involved in attendance of the patient will be reported
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