Radiographic classification and treatment of fibrous dysplasia of the proximal femur: 227 femurs with a mean follow-up of 6 years.

BACKGROUND: Research into the optimal treatment of fibrous dysplasia has been limited by the lack of an established classification system for the disease. The purposes of this study were to develop a radiographic classification for fibrous dysplasia of the proximal femur and to test this classification's intra- and interobserver reliability as well as the effectiveness of our treatments. METHODS: We retrospectively reviewed radiographs and computed tomography (CT) of 227 femurs from 206 patients with fibrous dysplasia. The radiographs were evaluated in the coronal plane for neck-shaft angle, varus deformity in the proximal femoral shaft, and distal juxtaarticular valgus deformity. CT was evaluated in the axial plane for destruction of cortex. Reduction of bone strength was defined as the thickness of the remaining cortex less than 50 % of the original on axial CT. Two senior orthopedists evaluated each radiograph and CT twice at 8-week intervals. Intra- and interobserver reliability testing was performed using the kappa statistic. Treatments were assessed through mid-term follow-up. RESULTS: The 227 femurs were classified into five reproducible types: type 1 (33 %), normal bone strength without angular deformity; type 2 (30 %), decreased bone strength without angular deformity; type 3 (12 %), isolated coxa vara with neck-shaft angle <120°; type 4 (11 %), isolated varus deformity in the proximal femoral shaft; and type 5 (14 %), coxa vara with varus deformity in the proximal femoral shaft. Intra- and interobserver kappa values were excellent, ranging from 0.85 to 0.88. Good clinical outcomes were achieved. CONCLUSIONS: This radiographic classification of fibrous dysplasia is reproducible and useful for describing and assessing this disease. The treatments based on this classification were effective.

Craniofacial fibrous dysplasia.

Despite recent advances in the understanding of the natural history and molecular abnormalities, many questions remain surrounding the progression and management of fibrous dysplasia (FD). In the absence of comorbidities, the expected behavior of craniofacial FD (CFD) is to be slow growing and without functional consequence. Understanding of the pathophysiologic mechanisms contributing to the various phenotypes of this condition, as well as the predictors of the different behaviors of FD lesions, must be improved. Long-term follow-up of patients with CFD is vital because spontaneous recovery is unlikely, and the course of disease can be unpredictable.

Florid osseous dysplasia: report of two cases and a review of the literature / Displasia ósea florida: reporte de dos casos y revisión de la literatura

Florid osseous dysplasia (FOD) is a benign condition of the jaws in which the normal architecture of bone is replaced by a fibrous tissue containing a variable amount of bone and cementum-like tissue. This lesion is most commonly seen in middle aged black women. FOD appears as dense, lobulated masses, often symmetrically located in the mandible, rarely in the maxilla. The lesion is usually asymptomatic and benign. However, a secondary infection may occur and its treatment can be difficult and complicated. This paper reports the case of two patients. The first one is a white woman aged 65 and the second one is a black woman aged 70, both diagnosed with FOD, revealed by secondary infections. The diagnosis was based on clinical and radiographic findings, as biopsy is contraindicated. Radiological and clinical features of FOD and its management will be also discussed on the basis of recent literature.

La displasia ósea florida (DOF) es una patología benigna del maxilar y mandíbula en la que se sustituye la arquitectura normal del hueso por un tejido fibroso que contiene una cantidad variable de tejido óseo y cementoide. Esta lesión es más frecuente en mujeres negras de edad media. La DOF aparece como una masa densa, lobulada, a menudo situada simétricamente en la mandíbula, rara vez en el maxilar. La lesión suele ser asintomática y benigna. Sin embargo, una infección secundaria puede ocurrir y su tratamiento puede ser difícil y complicarse. Este artículo reporta el caso de dos pacientes. La primera es una mujer blanca de 65 años y la segunda es una mujer negra de 70 años, ambas diagnosticadas con DOF, revelada por infecciones secundarias. El diagnóstico se basó en los hallazgos clínicos y radiológicos, ya que la biopsia está contraindicada. Las características clínicas y radiológicas de la DOF y su manejo son discutidos en base a la literatura reciente.

Cemento-osseous dysplasia in an elderly Asian male: a case report.

Cemento-osseous dysplasia is a disorder typically found in middle-aged black women. However, the present report describes a case in a 61-year-old Vietnamese male. Without proper pulp testing and diagnosis, the radiographic presentation can easily be misdiagnosed as periapical periodontitis. On the basis of pulp vitality, lack of clinical symptoms and radiographic features, the diagnosis in this case was periapical cemento-osseous dysplasia at the mixed stage, which generally requires no treatment. At the 18-month follow-up, the patient was still asymptomatic and none of the clinical signs had changed. This case highlights the importance of careful clinical examination, including a pulp vitality test, and of having an unbiased view of age, gender, and ethnicity when diagnosing this condition.

Expansive osseous dysplasia: report of 9 lesions in an African population sample and a review of the literature.

OBJECTIVES: The aim of this study was to report on the clinicopathologic features of osseous dysplasias (ODs) that manifest with gross expansion in a black South African hospital population sample. STUDY DESIGN: The files of 9 histology-verified expansive ODs in 8 patients were reviewed. The clinical records and radiographs were analyzed and compared with reports in the literature. RESULTS: The main complaint of all patients was related to expansion of the jaw. All except 1 were female, with ages between 26 and 71 years and sizes of the lesions ranging from 4 to 15 cm. No history of similar lesions in family members was obtained. All lesions occurred in the mandible and 6 crossed the midline. The expansive growth pattern was associated with persistence of radiolucent areas in the lesions which microscopically consisted of cellular fibro-osseous tissue. Resorption of mineralized deposits by osteoclasts was prominent in the radiolucent parts of the lesion. Maturation with enlargement of the radiodense component was associated with a decrease in osteoclast activity and the formation of lobular bone masses and confluent psammomatous mineralized deposits. CONCLUSION: We propose the term "expansive osseous dysplasia" for the rare albeit important clinical subcategory of ODs that manifest with progressive jaw expansion. Suspension of osteoclast activity plays an important role in the maturation of the lesions into dense mineralized masses.

Fibro-osseous lesions of the jaws: an analysis of 122 cases in Thailand.

BACKGROUND: There are no previously published reports on fibro-osseous jaw lesions in Thailand. The aims of this study were to determine the relative frequencies and the clinicopathologic features of these lesions in Thailand, and to compare these data with information available in the literature. METHODS: A total of 122 cases of fibro-osseous lesions of the jaws, out of a total of 4808 biopsy specimens, were collected from the files of the Department of Oral Pathology, Faculty of Dentistry, Mahidol University, Bangkok, Thailand, during a 34-year period from 1973 to 2006. Clinical data and histopathologic diagnoses were reviewed and analyzed. RESULTS: The most common was ossifying fibroma (50.8%), followed by fibrous dysplasia (42.6%). Ossifying fibroma most frequently occurred in the third and fourth decades of life (61.3%) and mostly involved the posterior region of the mandible (41.9%). Fibrous dysplasia was mostly seen in the second decade of life (40.4%). The maxilla was involved far more often than the mandible (53.8% and 6.2%, respectively), most common in the posterior region of the maxilla (28.8%). Both lesions presented as painless swellings (62.9% and 90.4%, respectively). Radiographically, ossifying fibroma mostly was mixed radiolucent-radiopacity (45.2%). Fibrous dysplasia mostly appeared as a radiopaque lesion (34.6%). CONCLUSIONS: The relative frequency of osseous dysplasia is underestimated because most lesions are not treated and the patients are kept under long-term follow-up without biopsy. The clinicopathologic features of ossifying fibroma and fibrous dysplasia in Thailand are identical to those in the literature.

Fibrous dysplasia in a Hong Kong community: the clinical and radiological features and outcomes of treatment.

OBJECTIVES: To evaluate the principal clinical and radiological features of a consecutive series of cases of fibrous dysplasia (FD) affecting a Hong Kong Chinese community and to determine their outcome by follow-up. METHODS: All cases were accompanied by appropriate radiography and were histopathologically confirmed as fibro-osseous lesions. RESULTS: The clinical and radiological presentations, differential diagnoses and outcomes of follow-up of 21 consecutive FD cases were reviewed. Females were slightly more frequently affected, but there was no gender predilection for either jaw. The mean age at first presentation was 25 years, which upon adjustment for prior awareness fell to 21 years. The main symptom was swelling (including enlargement of the jaw). The maxilla and mandible were affected in 9 and 13 cases, respectively; both jaws were affected in 1 case. One case was of McCune-Albright syndrome. The sole provisional diagnosis for all lesions was FD; most lesions affected all or almost half the affected jaw. 17 patients were followed-up for a mean of 9 years. CONCLUSIONS: This very detailed consecutive series followed up over many years, in which a number of cases recurred or were reactivated in adulthood, firmly dispells the notion that FD is a simple hamartoma in all cases. This community was quite amenable to really long-term follow-up.

Frequent immunoexpression of TGF-beta1, FGF-2 and BMP-2 in fibroblast-like cells in osteofibrous dysplasia.

Osteofibrous dysplasia (OFD) and fibrous dysplasia (FD) are both benign bone lesions which comprise the proliferation of fibroblast-like cells with bone formation, and these fibroblast-like cells have the phenotype of osteoprogenitor cells. The roentgenograph of OFD shows a heterogeneous osteolytic lesion with surrounding osteosclerosis, whereas FD is typically characterized by a rather homogeneous osteolytic lesion, or 'ground-glass appearance', with a smaller amount of surrounding osteosclerosis. Growth factors of transforming growth factor-beta1 (TGF-beta1), fibroblast growth factor-2 (FGF-2) and bone morphogenetic protein-2 (BMP-2) modulate bone differentiation. Expression of these growth factors was examined in the fibroblast-like cells of 16 cases of OFD and 16 cases of FD, immunohistochemically. TGF-beta1 in fibroblast-like cells was frequently expressed both in the OFD (16/16) and the FD (15/16) cases. The frequency of FGF-2 (16/16) expression and BMP-2 (13/16) expression in the fibroblast-like cells of OFD was higher than that of those [FGF-2 (8/16) and BMP-2 (6/16)] in the fibroblast-like cells of FD, with a statistical significance. These results seem to suggest that fibroblast-like cells of OFD have greater bone-forming ability than those of FD, and may explain the roentgenographic difference between OFD and FD and a difference in the nature of fibroblast-like cells between these two types of lesions.

Fractura patológica en edad pediátrica a causa de displasia fibrosa ósea / Pathological fracture at pediatric age due to osseous fibrous dysplasia

Se presenta un paciente de 13 años de edad, que a causa de un traumatismo sobre la cadera derecha sufrió fractura del extremo proximal del fémur. Se hace una revisión bibliográfica sobre clasificación actual, signos clínicos, radiográficos y anatomopatológicos sobre displasia fibrosa ósea, Se halló en los exámenes clínicos y paraclínicos del paciente que la zona fracturada comprendía hueso patológico. Se muestran las radiografías con los hallazgos obtenidos. Se realizó bopsia , curetaje de la lesión y se rellenó con material biocompatible. Constituye el primer caso reportado en Cuba de fractura en hueso patológico del extremo proximal del fémur, por displasia fibrosa ósea en edad pediátrica(AU)

Fractura patológica en edad pediátrica a causa de displasia fibrosa ósea / Pathological fracture at pediatric age due to osseous fibrous dysplasia

Se presenta un paciente de 13 años de edad, que a causa de un traumatismo sobre la cadera derecha sufrió fractura del extremo proximal del fémur. Se hace una revisión bibliográfica sobre clasificación actual, signos clínicos, radiográficos y anatomopatológicos sobre displasia fibrosa ósea, Se halló en los exámenes clínicos y paraclínicos del paciente que la zona fracturada comprendía hueso patológico. Se muestran las radiografías con los hallazgos obtenidos. Se realizó bopsia , curetaje de la lesión y se rellenó con material biocompatible. Constituye el primer caso reportado en Cuba de fractura en hueso patológico del extremo proximal del fémur, por displasia fibrosa ósea en edad pediátrica

Evaluación del desempeño clínico del radiólogo en el diagnóstico de la displasia fibrosa ósea: estudio de concordacia / Diagnostic concordance among radiologist in fibrous dysplasia cases

Introducción: la displasia fibrosa ósea es una entidad benigna que se caracteriza por la incapacidad para producir hueso lamelar maduro. Dependiendo de la proporción que exista entre hueso y tejido fibroso la apariencia radiológica es variable. Objetivo: establecer el grado, de acuerdo entre el consenso de dos radiólogos, y el diagnóstico definitivo, emitido por un grupo de expertos. Adicionalmente, describir los hallazgos radiológicos donde el diagnóstico fue acertado. Tipo de estudio: estudio de concordancia. Lugar del estudio: Instituto Nacional de Cancerología (Bogotá). Pacientes y métodos: 45 pacientes con sospecha por imágenes de displasia fibrosa ósea y diagnóstico definitivo por correlación clínica, radiológica e histológica, se realizaron pruebas de concordancia para evaluar el desempeño clínico de los radiólogos y la frecuencia de los hallazgos en los casos acertados. Resultados: los diagnósticos diferenciales fueron osteomielitis, encondroma solitario, encondromatosis múltiple, osteoblastoma, quiste óseo solitario y fibroma no osificante. La coincidencia entre el diagnóstico radiológico y el diagnóstico definitivo fue considerada sustancial o muy buena (kappa 0,6353). Las cinco patologías donde no hubo acuerdo diagnóstico fueron: la mastocitosis ósea poliostótica, el granuloma reparativo de células gigantes, el fibroma cemento-osificante, el fibroma no osificante y la osteomielitis crónica. La presentación radiológica del granuloma reparativo de células gigantes y el fibroma cemento-osificante son indistinguibles de la displasia fibrosa ósea, por lo tanto, se hace necesaria la confirmación histológica

Displasia fibrosa: reporte de 6 casos de variedad monostótica / Fibrous dysplasia: report of 6 cases of the monostotic variety

Los autores presentamos un enfoque clínico descriptivo de la displasia fibrosa, incluyendo nuestra experiencia en seis casos clínicos de diferente sexo y localización, detallando estudios y técnicas quirúrgicas realizadas

Displasia fibrosa: reporte de 6 casos de variedad monostótica / Fibrous dysplasia: report of 6 cases of the monostotic variety

Los autores presentamos un enfoque clínico descriptivo de la displasia fibrosa, incluyendo nuestra experiencia en seis casos clínicos de diferente sexo y localización, detallando estudios y técnicas quirúrgicas realizadas (AU)

Displasia osteofibrosa: experiencia de 10 años / Osteo-fibrous displasia: 10 years experience

La displasia ósteo-fibrosa es un tumor benigno poco frecuente en cabeza y cuello. Se localiza de preferencia en el hueso maxilar y afecta, con cierta frecuencia, las cavidades perinasales. Habitualmente asintomática, su tratamiento es quirúrgico cuando produce importante deformidad facial o síntomas comprensivos vásculo-nerviosos orbitarios o rinosinusales. Se presenta la experiencia del Servicio de Otorrinolaringología del Hospital Clínico de la Universidad de Chile, con un total de 7 casos en un período de 10 años. Se detalla los motivos de consulta, localización y tratamiento quirúrgico realizado en estos pacientes. Se hace hincapié en el enfoque multidisciplinario y en la importancia del seguimiento a largo plazo para manejar las recidivas y una eventual malignización de estos tumores

Florid cemento-osseous dysplasia in a young Chinese man. Case report.

Florid cemento-osseous dysplasia refers to a group of fibro-osseous lesions which are exuberant, multiquadrant and arise from the tooth-bearing area of the jaws. It is classically described as a condition occurring almost exclusively in middle-aged black women. A case of florid cemento-osseous dysplasia occurring in a young Chinese male is reported which was rare in regard to race and sex. This 20 year old Chinese man presented with huge symmetrical bony lesions in all four quadrants of the jaws. Clinical presentation, radiological findings and histological features of the excised specimens are described. Treatment of the lesions was unusual. Curettage was first done with minimal benefit and it was followed by mandibular recontouring to improve facial appearance. The outcome of these procedures will be discussed.

Criteria and quantification of fibrous dysplasia on MDP scanning.

Eleven patients with histologically proven fibrous dysplasia (biopsy) were imaged with 3-phase bone scanning, using 99mTc-MDP. Ten had monostotic and one had polyostotic fibrous dysplasia. The perfusion (phase I) and uptake (phase III) of monostotic lesions was compared to the normal contralateral side and the rest of the skeleton was inspected for other abnormalities. Lesions presented with slightly increased perfusion (1.65 normal +/- 0.907) and markedly increased uptake of tracer (6.0 normal +/- 2.58). Other features that were noted are high spinal and scapula and poor renal tracer uptake.

Oncogenic osteomalacia: a clinicopathologic study of 17 bone lesions.

Oncogenic osteomalacia is an unusual and rare clinicopathologic syndrome characterized by mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities consisting of hypophosphatemia, normocalcemia, and increased levels of alkaline phosphatase. We collected from the Mayo Clinic files and from our consultation files the records for 17 cases of osteomalacia associated with bone lesions. There were five cases of fibrous dysplasia, three of hemangiopericytoma, and two of phosphaturic mesenchymal tumor. There was one case each of osteosarcoma, chondroblastoma, chondromyxoid fibroma, malignant fibrous histiocytoma, giant cell tumor, metaphyseal fibrous defect, and hemangioma. In this study we can figure out that the most common characteristic histologic features of our cases were hemangiopericytomatous vascular proliferation, fine lace-like stromal calcification, and stromal giant cells. In most of the cases, the clinical and biochemical symptoms and signs resolved soon after complete resection of the lesion. When the lesion recurred or metastasized, the symptoms and signs also recurred.