Fibrous Dysplasia

Fibrous dysplasia is a developmental benign bone lesion characterised by the replacement of normal bone by excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae. Fibrous dysplasia of the jaw are believed to be benign, self-limiting, nonencapsulated mainly occurring in young subjects. Although, jaw lesions of fibrous dysplasias are usually monostotic, they may occasionally be
part of a polyostotic process. Monostotic fibrous dysplasia of the maxilla is the most common site of involvement in the facial bones and accounts for approximately 70% of those with facial involvement. We report, a case with a slow growing swelling in relation to right and left side of face.

PURPOSE OF THE STUDY The aim of the study was to analyze primary malignang tumors and tumor-like lesions of long bones, in relation to their localization, characteristics and distribution in age groups, in children and young adults, and to assess the role of radiog- raphy and magnetic resonance imaging (MRI) in their diagnosis. MATERIAL Sixty-four patients, aged between 3 and

Fibrous dysplasia is a developmental disorder of the bone that originates from a genetic defect disturbing the osteogenesis leading to the replacement of normal bone with the excess proliferation of fibrous tissue. It can be associated with hyperpigmentation of the skin and endocrine disorders. Fibrous dysplasia can manifest in a monostotic form affecting one bone or in a polyostotic form involving several bones. Approximately 30% of monostotic forms are observed in the maxilla and the mandible. It frequently appears in the posterior region and is usually unilateral. It is found in teenagers and could become static after adulthood. Patients can present with swelling, facial asymmetry, pain, or numbness on the affected side. Treatment modalities vary between conservative surgical treatment, radical surgical approach, and medical treatment based on bisphosphonates. Here, we present a case of a monostotic form of fibrous dysplasia affecting the posterior left region of the mandible in ...

Adamantinoma of the long bones is a rare, low-grade malignancy with a marked predilection for the tibia and is usually seen in patients during the second to fifth decades of life. Adamantinomas have also been reported in children, but the histological pattern in this age group is different from that seen in adults. We report a case of adamantinoma of

Adamantinoma of the long bones is a rare, low-grade malignancy with a marked predilection for the tibia and is usually seen in patients during the second to fifth decades of life. Adamantinomas have also been reported in children, but the histological pattern in this age group is different from that seen in adults. We report a case of adamantinoma of

Fibrous dysplasia presents in two forms: monostotic and polyostotic. Both forms are more widespread among children and juveniles and may result in facial asymmetry. Neoplastic bone lesions, localized over the root apices and mimicking periapical pathosis, have been observed. Irregular pulp morphology not previously reported is described in the following case report. A 14-year-old boy diagnosed with fibrous dysplasia was referred for endodontic treatment as the result of a necrotic pulp in an upper molar. A periapical lesion was diagnosed when the tooth was intact. After complex root canal treatment the periapical lesion healed.

RESUMEN El término displasia fibrosa hace referencia a un conjunto de lesiones óseas benignas que se caracterizan por la sustitución del tejido óseo normal por tejido conectivo. Se presenta el caso de una paciente afectada de displasia fibrosa poliostótica de ...

Polyostotic fibrous dysplasia is a skeletal disease that results from somatic activating mutations in the gene GNAS in skeletal stem cells, leading to proliferation of immature osteogenic cells with replacement of normal marrow and bone with fibro-osseous tissue. Lesions may cause bone deformity or fracture. In the surgical care of polyostotic fibrous dysplasia, the role of grafting and the optimal grafting material are not clear. The purpose of this study was to evaluate the long-term survival of bone-grafting procedures in subjects with polyostotic fibrous dysplasia over time. The operative reports and radiographs of a cohort of subjects with polyostotic fibrous dysplasia followed in a natural history study were reviewed. Twenty-three subjects (mean age at the time of enrollment, thirteen years [range, two to forty years]) with fifty-two bone-grafting procedures had a mean follow-up time of 19.6 years (range, twenty-nine months to forty-seven years). Kaplan-Meier life table estima...

Mazabraud’s syndrome is a rare disorder, the main characteristics of which are fibrous dysplasia of bone associated with intramuscular myxomas. The metabolic characteristics of intramuscular myxomas, associated with fibrous dysplasia, have not previously been described with 18F-FDG-PET. Our case demonstrates that there is low, but not insignificant uptake associated with these intramuscular myxomas, with a standardized uptake value (SUV) range between 1.3 and 2.6. As such, this entity merits consideration when evaluating hypoattenuating intramuscular masses, particularly in the context of fibrous dysplasia.

Fibrous dysplasia (FD) patients sometimes suffer from concomitant hypophosphatemic rickets/osteomalacia, resulting from renal phosphate wasting. It was recently reported that FD tissue in the patients with McCune–Albright syndrome (MAS) expressed fibroblast growth factor-23 (FGF-23), which is now known to be as a pathogenic phosphaturic factor in patients with oncogenic osteomalacia and X-linked hypophosphatemic rickets. Since it remains controversial whether serum

Background: Stigma, both enacted and internalized, is part of the illness experience of many chronic conditions / diseases and has been found to increase psychological distress, lower self-esteem, and impact social engagement lowering quality of life (QOL). Stigma among pediatric patients is of particular concern due to its potential impact on identity formation. Using patient data from the online FD/MAS Alliance Patient Registry (FDMASAPR), this study seeks to 1) determine levels of enacted and self-stigma in a pediatric population of fibrous dysplasia (FD) / McCune Albright syndrome (MAS) patients and 2) to explore the relationship between stigma and anxiety and depression. Methods: This is a cross sectional analysis of deidentified self-report data from 18 pediatric patients. Key analytic variables include the Neuro-QOL stigma short form, the Hospital Anxiety and Depression Scale (HADS), diagnostic category and craniofacial involvement, and select demographics. Sample means and score distributions are examined. Bivariate relationships between stigma, anxiety and depression and patient's personal and medical characteristics are established through analysis of variance and correlation. Results: Composite stigma levels for FD/MAS pediatric patients were comparable to those of children with multiple sclerosis, epilepsy, and muscular dystrophy. Self-stigma was more frequently reported than enacted/felt stigma, but few patients indicated complete freedom from either type of stigma. Diagnosis was significantly related to selfstigma. Significant bivariate relationships were found between depression and enacted/felt and self-stigma and between anxiety and self-stigma. Conclusions: This study establishes the illness experience of pediatric patients with FD / MAS is impacted by stigma and suggests they should be regularly screened for stigma and psychological distress. It supports the integration of clinical psychologists/ therapists in regular patient care, referral of families to advocacy organizations, and indicates that rare disease patient registries can be a useful tool in efforts to improve the QOL of patients.