Cases reported "Fibrous Dysplasia of Bone"

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1/25. Malignant spindle cell tumor arising in the mandible of a patient with florid osseous dysplasia.

    Florid osseous dysplasia is a non-neoplastic condition of the alveolar processes of the jaws characterized by the replacement of multiple foci of bone by fibrous connective tissue, accompanied by gradual deposition of cementum, bone, or both. The lesions are not associated with inflammatory diseases of the dental pulp or periodontal tissues. In fully developed florid osseous dysplasia, there are multiple lobulated masses in the alveolar bone bilaterally in the mandible and sometimes in the maxilla. This is the first report of a malignancy originating within the jaws of a patient with florid osseous dysplasia. A spindle cell malignancy was diagnosed in the mandible of a 54-year-old black woman whose jaw was affected by florid osseous dysplasia bilaterally. Despite extensive surgery and radiotherapy, the patient died 20 months after diagnosis of the malignancy.
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2/25. Florid osseous dysplasia of the mandible: report of a case.

    In 1986, a 60-year-old African American woman visited the Marquette University School of dentistry with a complaint of a vague, dull pain in her lower left quadrant. tooth No. 19 was extracted 10 years earlier because of extensive decay, and tooth No. 18 had received root canal therapy. A panoramic radiograph revealed the presence of ill-defined, multilocular, mixed (radiopaque-radiolucent) lesions present throughout the lower jaw. She was treated with antibiotics and scheduled for follow-up visits. When the symptoms persisted, tooth No. 18 was re-treated with root canal therapy and a representative biopsy was taken from the left mandibular area. The biopsy showed the presence of chronic osteomyelitis. The patient was treated with antibiotics and was scheduled for periodic check-up visits. In February 1995, she returned with the same symptoms in the left mandible. A panoramic radiograph showed persistence of the mixed radiopaque-radiolucent lesions throughout her mandible; however, the mass on the left side was more radiopaque and had assumed a "cotton wool" appearance. tooth No. 18 was extracted and a biopsy was taken from the area. After correlating the clinical behavior, radiographic appearance, and histopathologic features, a diagnosis of florid osseous dysplasia with osteitis was made. This case represents a classic example of the difficulty in diagnosing fibro-osseous lesions using radiographic interpretation alone and the need to correlate the clinical, radiographic, and histopathologic features to reach a diagnosis. Additionally, the present case clearly shows treatment problems of an otherwise self-limiting condition when secondary involvement with osteomyelitis is also present. A brief description of the conditions that were included in the differential diagnosis and their management is presented.
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3/25. Gigantiform cementoma: clinicopathologic presentation of 3 cases.

    Gigantiform cementoma is a rare, benign fibro-cemento-osseous disease of the jaws, seen most frequently in young girls. Radiographically, it typically presents as multiquadrant, expansile, mixed radiolucent-opaque lesions that cross the midlines of the jaws. Although cases with a familial pattern are noted in a few publications, sporadic cases have been reported without a family history. The term gigantiform cementoma has been used interchangeably with designations of other fibro-osseous entities, yet its application should be restricted by the criteria defined herein. In this article, we report 3 cases of gigantiform cementoma. Clinical, radiographic, and microscopic features of these lesions are presented, along with criteria to differentiate gigantiform cementoma from other fibro-osseous diseases of the jaws. A possible pathogenetic mechanism and treatment recommendations are discussed.
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4/25. Concomitant focal fibrocartilaginous dysplasia of the tibia and eosinophilic granuloma of the jaw in a child.

    This 2-year-old child presented with concomitant eosinophilic granuloma of the lower jaw and focal fibrocartilaginous dysplasia of the right tibia. Her eosinophilic granuloma was diagnosed on the basis of the clinical picture, imaging studies and the characteristic histologic appearance. Focal fibrocartilaginous dysplasia was revealed incidentally during the eosinophilic granuloma staging process. After chemotherapy, all signs of eosinophilic granuloma subsided, but focal fibrocartilaginous dysplasia remained without signs of clinical or radiographic progression. The importance of differentiating these two conditions is stressed in order to avoid ineffective and inappropriate treatment of focal fibrocartilaginous dysplasia.
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5/25. cherubism in siblings: a case report.

    cherubism is a non-neoplastic bone disease characterized by clinically evident bilateral, painless enlargements of the jaws that are said to give the patient a cherubic appearance. cherubism may appear in solitary cases or in many members of the same family, often in multiple generations. On radiography, the lesions exhibit bilateral multilocular radiolucent areas. Histopathologic evaluation reveals proliferating fibrous connective tissue containing numerous multinucleated giant cells. Since the first description of this condition in 1933, almost 200 cases have been reported. We describe cherubism in 2 siblings and briefly review the literature on this subject.
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6/25. Feasibility of osteotomies in fibrous dysplasia of the jaws.

    Two examples are presented of osteotomies performed simultaneously with surgical recontouring in cases of fibrous dysplasia of the jaws. Osteotomies are necessary when a coexistent discrepancy in jaw relationship is present. This provides a functional as well as aesthetic result. Rigid miniplate fixation was utilised, and dysplastic bone healing was uneventful, and found clinically not to differ from that of normal bone. osteotomy is thus feasible in dysplastic bone, and may complement recontouring in achieving a better result.
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7/25. Fibrous dysplasia of the jaws: a case report.

    This article reviews the present clinical, radiographic and histologic features of fibrous dysplastic lesions of the jaws. A case is presented of an apparently reactivated fibrous dysplastic jaw lesion, with evidence of cystic degeneration, in a 33-year-old female school-teacher who was first seen at the Department of Dental Surgery, University of Nairobi in March 1986. The dilemma in the management of fibrous dysplasia is reviewed and discussed. It is noted that while conservative surgical procedures remain the treatment of choice, situations arise where tremendous tissue activity would require early intervention by numerous cosmetic surgical shaving procedures. The present case, however, offers a unique management challenge due to the occurrence of extensive cystic degeneration in the mandible.
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8/25. Fibrous dysplasia and cherubism as an hereditary familial disease. Follow-up of four generations.

    Five cases of dysplasia of the jaws in one family which has been under our observation since 1970 are reported. The disease appeared as a mixed display of jaw lesions, in some members as fibrous dysplasia and in others as cherubism. We were able to trace the disorder through an unbroken line of four generations, and thus to demonstrate autosomal dominant inheritance. cytogenetic analysis performed on three members of this family revealed a significantly increased rate of chromosomal breakage.
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9/25. Fibrous dysplasia of the mandible and sphenoid bones: report of case.

    A case of fibrous dysplasia of the mandible has been presented in which further diagnostic procedures showed the presence of a second and potentially more dangerous lesion. The literature was reviewed with the purpose of bringing attention to the still confused state of fibro-osseous lesions of the jaws and skeleton. The relationship of the ossifying fibroma and fibrous dysplasia was explored in relative depth. Fibrous dysplasia is a usually benign fibro-osseous abnormality of bone that may occur as monostotic, polyostotic, or craniofacial disease or as a part of a syndrome. Its nosology is confusing and its etiology is still unknown.
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10/25. Giant fibrous dysplasia of the mandible: surgical management.

    Giant fibrous dysplasia of the mandible (GFDM) represents a far advanced lesion which may be monostotic, polyostotic, a component of craniofacial fibrous dysplasia, a component of Albright's syndrome, or associated with other endocrinopathies. The current philosophy is to consider fibrous dysplasia of the mandible (FDM) as one of a group of lesions classified as fibro-osseous lesions of the jaws. The natural course and manifestations in the mandible result in GFDM if they are unabated by curative therapy or if stabilization or regression does not occur. A rational therapeutic approach depends upon the extent of the disease at the time of diagnosis. The earlier in the disease process that the diagnosis is made, the more likely that a curative surgical procedure may be undertaken. Complete resection of the diseased mandible should be performed when feasible. A lesser surgical procedure has no curative potential and is performed only as a palliative, cosmetic delaying procedure.
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