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      Jawbone fibrous dysplasia: retrospective evaluation in a cases series surgically treated and short review of the literature

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          Abstract

          Background and aim of the work.

          Fibrous dysplasia is a fibro-osseous osteopathy in which the normal bone architecture is replaced by fibrous tissue and non-functional trabeculae-like osseous structures. In head and neck area monostotic or polyostotic lesions cause a progressively expanding destructive bone swelling producing cosmetic deformities and functional impairments. The aim of this article is to present a retrospective review of a clinical case series with pathologically confirmed jawbone fibrous dysplasia for over an 8-year-period.

          Material and Methods.

          Clinical presentation and radiographic features of fibrous dysplasia affecting the jawbone skeletal area, surgical procedures performed including the reconstructive methods employed and clinical outcomes were analysed for each patient.

          Results.

          Seven cases were classified as having monostotic fibrous dysplasia while the others four cases were classified as having polyostotic form. The mandible was most commonly involved. The most common presenting features included marked facial deformity, intraoral bulging, malocclusion and dental alterations. Aesthetic and/or functional impairments were the major indications for surgical treatment in all the patients of this series. Six patients underwent bone remodelling while in the remaining cases subtotal or total resection was performed. Bone reconstruction by means of autologous free bone grafts or revascularized free bone flaps was made in three cases.

          Conclusion.

          The choice of the tailored therapeutic approach should be evaluated according the patient’s age, rate of growth, anatomic location, type of involvement and the presence or not of functional disturbances and cosmetic alterations. Surgery remains the best therapeutic option.

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          Most cited references57

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          Malignancies in fibrous dysplasia.

          Malignancies in fibrous dysplasia are rare. Most cases have been published as single case reports. The role of radiation therapy in the occurrence of sarcoma in fibrous dysplasia is still controversial. The Mayo Clinic files were reviewed, including Mayo Clinic cases and consultation cases, to collect all cases of sarcomas arising in fibrous dysplasia. Among 1122 cases with a histologic diagnosis of fibrous dysplasia, 28 cases of sarcoma were found. These 28 cases included 16 Mayo Clinic cases and 12 consultation cases. The sarcomas occurred in 19 cases of monostotic fibrous dysplasia and 9 cases of polyostotic disease (only 1 of Albright's syndrome). The most common histotype was osteosarcoma (19 cases), followed by fibrosarcoma (5 cases), chondrosarcoma (3 cases), and malignant fibrohistiocytoma (1 case). Of the 28 patients, 13 (46%) had received radiation therapy before the sarcoma developed. Most of these sarcomas occurred in the craniofacial bones (13 cases) or in the proximal femur (7 cases), followed by the humerus, pelvis, tibia, and scapula. Prognosis was poor. Sarcomas may arise with or without radiation. Early diagnosis and adequate treatment may lead to improved prognosis.
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            Fibro-osseous lesions of the jaws.

            In the first volume of the Journal of Oral Surgery, Dr G. Victor Boyko presented a case of osteofibroma of the mandible associated with leontiasis ossea of the skull (Boyko GV: J Oral Surg 1:100, 1943). The patient was a 32-year-old white woman who had complaints of right mandibular enlargement and a prominence of the right frontal and temporal areas of uncertain duration in August 1939. Mandibular radiographs showed an area of reduced radiodensity. Skull films showed a marked increase in density of the inferior part of the right temporal region and of the frontal bone, with evidence of both bone destruction and proliferation in the inferior frontal and orbital regions. A mandibular biopsy was reported as osteofibroma. The patient was kept under observation for 8 months after the biopsy, with little change in her condition. A course of deep x-ray therapy was then delivered to the mandibular lesion. She subsequently developed a pathologic fracture that was treated by maxillomandibular fixation. The fracture stabilized and at the last clinical examination, in June 1942, the patient had a union with good functional occlusion and was reported to be in good mental and physical condition.
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              Fibrous dysplasia of bone: craniofacial and dental implications

              Fibrous dysplasia (FD) is a rare bone disease caused by postzygotic somatic activating mutations in the GNAS gene, which lead to constitutive activation of adenylyl cyclase, and elevated levels of cyclic AMP, which act on downstream signaling pathways, and cause normal bone to be replaced with fibrous tissue and abnormal (woven) bone. The bone disease may occur in one bone (monostotic), multiple bones (polyostotic), or in combination with hyperfunctioning endocrinopathies and hyperpigmented skin lesions (in the setting of McCune-Albright Syndrome). FD is common in the craniofacial skeleton, causing significant dysmorphic features, bone pain, and dental anomalies. This review summarizes the pathophysiology, clinical findings and treatment of FD, with an emphasis on the craniofacial and oral manifestations of the disease.
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                Author and article information

                Journal
                Acta Biomed
                Acta Biomed
                Acta Bio Medica : Atenei Parmensis
                Mattioli 1885 (Italy )
                0392-4203
                2531-6745
                2021
                13 October 2020
                : 92
                : 1
                : e2021018
                Affiliations
                [1 ] Oral and Maxillo-Facial Department, Provincial Rovigo Hospital, Italy
                [2 ] Department of Medical and Surgical Sciences and Advanced Technologies “GF Ingrassia”, ENT Section; University of Catania- Italy
                [3 ] Otorhinolaryngology Department, Provincial Rovigo Hospital (Italy)
                [4 ] Department of Neurosciences, Section of Otolaryngology and Regional Centre for Head and Neck Cancer, University of Padova, Treviso, Italy
                [5 ] Department of Otorhinolaryngology-Head and Neck Surgery, Cattinara Hospital, University of Trieste, Trieste, Italy
                [6 ] Allergy Clinic, Casa di Cura Villa Montallegro, Genoa, Italy
                [7 ] Department of Neurosciences, Section of Otolaryngology and Regional Centre for Head and Neck Cancer, University of Padova, Treviso, Italy
                [8 ] Department of Surgery, Oncology and Gastroenterology, Section of Oncology and Immunology, University of Padova, Padova, Italy.
                Author notes
                Correspondence: Dott. Maniaci Antonino ENT Department of University of Catania 95100, Via Santa Sofia Catania Italy, Phone: +39.0953781103 E-mail: tnmaniaci29@ 123456gmail.com
                Article
                ACTA-92-18
                10.23750/abm.v92i1.9904
                7975924
                33682826
                58081e5f-e725-476c-a4e6-c27dbdf0b4d9
                Copyright: © 2020 ACTA BIO MEDICA SOCIETY OF MEDICINE AND NATURAL SCIENCES OF PARMA

                This work is licensed under a Creative Commons Attribution 4.0 International License

                History
                : 25 May 2020
                : 16 June 2020
                Categories
                Original Article

                fibrous dysplasia,jawbone,bone remodelling,craniofacial dysmorphisms

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