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PubMed1985

Fibrous dysplasia of bone and concomitant dysplastic changes in the dentin.

Fibrous dysplasia of bone and dentinal dysplasia have not been classified as related conditions. However, the present report describes a patient with both polyostotic fibrous dysplasia and dysplastic changes in the dentin. One bone lesion was first discovered on the right side of the mandible on a routine roentgenogram. There was an atypical radiolucency apical to a short-rooted molar with an obliterated pulp. Additional lesions were found on the left side of the mandible in the maxilla, in the frontal and occipital bones, in the ilium, in the proximal ulnae, and in the ribs. All lesions demonstrated an increased uptake of Tc99. A biopsy specimen of bone from the right side of the mandible showed small calcified islands in a cell-rich connective tissue. Microscopic analysis of the right first molar showed irregularly shaped dentin with the dentinal tubules arranged in a whorl-like fashion surrounding an almost completely obliterated pulp chamber and canal. Healing after tooth extraction and after the bone biopsy was unremarkable. After 6 months the alveolus was reorganized and the bone patterns appeared normal, although the radiolucent areas inferior to the teeth remained unchanged. The concomitant occurrence of dysplastic changes in bone and teeth may be a sign of a generalized defect in the mesenchymal hard-tissue-forming cells.

Adult↗
PubMedDate not supplied

Fibrous dysplasia: a review of the disease and its manifestations in the temporal bone.

Fibrous dysplasia is a fairly common, localized misdifferentiation of the bone-forming mesenchyme affecting a single or many bones, in which skeletal aberrations represent the cardinal feature, but in which certain endocrinopathies, abnormal pigmentation of skin and mucous membrane, and occasionally other abnormalities form part of the entire disease process. The craniofacial skeleton is one of its predilective sites and therefore the temporal bone may become involved. In such instances the disease manifests itself with 1) progressive loss of hearing, 2) increasing obliteration of the external ear canal, and 3) enlargement and distortion of the temporal bone. The first part of this communication is concerned with a review of the clinical and pathological aspects of the disease in general. The second part is concerned with a discussion of the clinical manifestations in the temporal bone, based upon analysis of the entire literature and the authors' personal experience. The indications for surgical intervention include 1) maintenance of a normal outer ear canal, 2) preservation of cochlear and vestibular function, and 3) prevention of secondary complications.

Adolescent↗
PubMed1997

Fibrous dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation.

In addition to café-au-lait pigmentation patterns and hyperendocrinopathies, fibrous dysplasia of bone is a major finding in the McCune-Albright syndrome. Activating missense mutations of the Gs alpha gene leading to overactivity of adenylyl cyclase have been identified in patients with McCune-Albright syndrome, but the mechanism leading to the specific development of fibrous dysplasia in bone has not been elucidated. By means of specific peptide antisera and reverse transcriptase polymerase chain reaction in situ hybridization, we show that expression of Gs alpha and its mRNA is critically up-regulated during maturation of precursor osteogenic cells to normal osteoblast cells and that this pattern of expression is retained in fibrous dysplasia. A functional characterization of fibrous dysplastic tissues revealed that the fibrotic areas consist, in fact, of an excess of cells with phenotypic features of pre-osteogenic cells, whereas the lesional bone formed de novo within fibrotic areas represents the biosynthetic output of mature but abnormal osteoblasts. These cells are noted for peculiar changes in cell shape and interaction with matrix, which were mimicked in vitro by the effects of excess exogenous cAMP on human osteogenic cells. Osteoblasts involved with the de novo deposition of lesional bone in fibrous dysplasia produce a bone matrix enriched in certain anti-adhesion molecules (versican and osteonectin), and poor in the pro-adhesive molecules osteopontin and bone sialoprotein, which is in contrast to the high levels of these two proteins found in normal de novo bone. Our data indicate the need to reinterpret fibrous dysplasia of bone as a disease of cells in the osteogenic lineage, related to the effects of excess cAMP on bone cell function. They further suggest that a critical, physiological, maturation-related regulation of Gs alpha levels makes cells in the osteogenic lineage a natural target for the effects of mutations in the Gs alpha gene and may provide a clue as to why bone itself is affected in this somatic, mutation-dependent disease.

Adolescent↗
PubMed2000

Parathyroid hormone-related protein in the aetiology of fibrous dysplasia of bone in the McCune Albright syndrome.

OBJECTIVE: Fibrous dysplasia, observed in bone lesions in the McCune Albright syndrome (MAS), is thought to result from abnormalities in cells of the osteogenic lineage associated with over-activation of the cAMP signalling pathway in affected cells. The aim of this study was to investigate the role of parathyroid hormone-related protein (PTHrP) in the aetiology of MAS, and to determine a possible therapeutic role for 1,25-dihydroxy vitamin D(3) (1,25(OH)(2)D(3)). DESIGN: The effects of 1,25(OH)(2)D(3) on PTHrP production and mRNA expression were determined in vitro. 1,25(OH)(2)D(3) therapy was administered to three patients with MAS. PATIENTS: Clinical data from four MAS patients (MAS1, 2, 3 and 4), and in vitro studies using bone from three MAS patients (MAS1, 2, and 3), are presented. MEASUREMENTS: Immunoradiometric assay and low-cycle number reverse transcriptase-linked PCR were used to determine PTHrP production and mRNA expression in vitro. Standard clinical biochemistry was recorded pre and post commencement of 1,25(OH)(2)D(3) treatment. RESULTS: We report the elevated secretion of PTHrP, and a concomitant rise in PTHrP mRNA expression, in cultured osteoblasts from three MAS patients. Treatment with 1,25(OH)(2)D(3) produced a dose-dependent decrease in PTHrP protein secretion and mRNA expression. Marked improvement in bone biochemistry in MAS1, 2 and 3 post treatment with 1,25(OH)(2)D(3) is documented. CONCLUSION: This study provides the first evidence suggesting that PTHrP may contribute to the aetiology of fibrous dysplasia in MAS. In addition, the therapeutic administration of 1,25(OH)92)D(3) may provide clinicians with an important new regime for symptomatic relief of bone pain and fracture in some patients with MAS.

Adolescent↗
PubMed1997

[Fibrous dysplasia of the maxillofacial bones. Clinical considerations].

OBJECTIVE: After a wide survey of the literature, the results of a clinical-statistic study carried out on twelve cases of maxillo-facial bones fibrous dysplasia, diagnosed and treated at the Oral and Maxillo-Facial Surgery Department of the II University of Naples, are reported. EXPERIMENTAL ASSAY: Retrospective study. MATERIALS AND METHODS: All the patients examined showed fibro-osseus lesions involving the mandible in eight cases, the maxilla in three cases and the frontal bone in one case. Seven patients were females and five males, aged between nine and seventy-two years. The surgical treatment was carried out on eleven patients: four cases were treated by complete surgical excision of the fibro-osseous lesions and seven cases by a conservative removal of the portion of the lesion contributing to the bone deformity. RESULTS AND CONCLUSIONS: All cases examined involved maxillo-facial skeleton structures as a sole localization, confirming that the polyostotic forms are more infrequent than the maxillary monostotic ones. The distribution of the cases according to age classes and lesion localization showed a higher incidence of fibrous dysplasia in the third decade of life and a clear predilection for the mandible. The observation of active lesions in old age shows that the disease may evolve also after the growth. The cases treated by radical excision of the lesions have not been followed from relapse; on the contrary three of the seven cases treated by conservative excision relapsed after a few years.

Adolescent↗
PubMed2003

Is McCune-Albright syndrome overlooked in subjects with fibrous dysplasia of bone?

OBJECTIVE: McCune-Albright syndrome (MAS) is characterized by a clinical triad of endocrinopathies, café au lait pigmentation, and polyostotic fibrous dysplasia of bone. We hypothesized that children diagnosed with fibrous dysplasia are not routinely being evaluated for coexisting endocrine dysfunction or MAS. Our objective was to prospectively screen subjects with fibrous dysplasia for endocrine disease and G(s)alpha gene (GNAS1 )-activating mutations. STUDY DESIGN: Nine subjects who presented with fibrous dysplasia and were followed in orthopedic clinics were evaluated for other manifestations of MAS. Genomic DNA was isolated from blood, and mutation analysis of GNAS1 was performed. RESULTS: On physical examination, 5 of 9 subjects were found to have café au lait pigmentation. Three of 9 subjects had TSH levels below the normal range. One of these subjects was found to have hyperthyroidism and was treated by total thyroidectomy. GNAS1 mutations were identified in 5 of 9 subjects with either monostotic or polyostotic fibrous dysplasia of bone. CONCLUSIONS: We conclude that a substantial proportion of children being followed for fibrous dysplasia of bone have unrecognized clinical and laboratory features of MAS. These children are at risk for endocrinopathy and should be screened accordingly.

Adolescent↗
PubMedDate not supplied

[Surgical reconstruction of fibrous dysplasia of bone in long-term follow-up].

PURPOSE: To examine how different operative measures influence the surgical outcome in patients with fibrous dysplasia of bone. METHODS: 118 dysplastic fibrous lesions of bone were surgically treated and reviewed in 70 patients between 1983 to 1993 (eleven years) with a median follow-up of six and a half years. Surgery consisted of intralesional curettage in 93 and marginal en bloc resection in 25 lesions. Bony defects were reconstructed with autogenous iliac crest graft in 55 lesions, with autogenous fibula graft in 9, with homologous bone chips in 28, and 5 times with a homologous fibula graft from the bone bank. In 33 lesions the entire defect was filled with polymethylmethacrylate. Osteosynthesis was performed in 41 patients. RESULTS: Recurrences requiring surgical revision were observed in 26 of 74 primary lesions (= 35% overall recurrence rate) at a mean 123.6 weeks postoperatively. The most frequent primary and recurrence location was the proximal femur (85% revision rate). 69% of all recurrences occurred under the age of 20. After intralesional curettage the reoperation rate was 32% and after marginal resection 8%. After reconstruction with autogenous iliac crest graft recurrence rate was 36%, after autogenous fibula graft 55%, after homologous bone chips 18%, after polymethylmethacrylate 9% and allograft fibula reconstruction showed no recurrences. A combined stable osteosynthesis bridging the fibrous osseous defect significantly reduced the revision rate to 3% (p = 0.01). CONCLUSION: Intralesional curettage and reconstruction with autogenous iliac crest graft in fibrous dysplasia of bone leads to a high recurrence rate. Reconstruction with cortical grafts or bone chips from the bone bank, if necessary in combination with a durable osteosynthesis in mechanically demanding locations, or solely bone cement in mechanically less demanding areas, reduces the revision rate in patients with monoostotic and polyostotic fibrous dysplasia.

Adolescent↗
PubMed1994

Long-term effects of intravenous pamidronate in fibrous dysplasia of bone.

Nine patients with symptomatic and severe fibrous dysplasia were treated with intravenous pamidronate (60 mg per day over 3 days every sixth month), and were followed up for 18-48 months. The major effect was decreased bone pain (complete remission in 12 of 14 sites). Radiological changes were seen in four patients, with thickening of cortices, refilling of osteolytic lesions, or both. The initial increased bone remodelling was reduced, as shown by decrease of raised serum alkaline phosphatase and urinary hydroxyproline. The treatment was well tolerated, but a 13-year-old patient showed widening of knee growth-plates which is consistent with a transient mineralisation defect.

Adolescent↗
PubMed1994

[Fibrous dysplasia of bone].

The natural history, clinical and radiological manifestation and surgical treatment of fibrous dysplasia of bone have been presented.

Fibrous Dysplasia of Bone↗
PubMed2001

Fibrous dysplasia of bone: management and outcome of 20 cases.

BACKGROUND AND OBJECTIVES: Fibrous dysplasia of bone is difficult to manage because of its variable clinical course with many different methods of treatment reported. Therefore we report on our experience. METHODS: We reviewed a series of 20 patients with 32 lesions included. The average age at the time of diagnosis was 32 years for monostotic disease, 26 years for polyostotic disease, and 3 years for McCune-Albright syndrome. The median follow-up period was 6 years. Functional and radiographic outcomes were scored. RESULTS AND CONCLUSIONS: Monostotic disease mostly presented with a circumscribed lesion and monitoring was often sufficient. Symptomatic circumscribed lesions showed satisfactory outcome when treated with curettage, cryosurgery and bone grafting. Lesions of the extended type were most of all seen in polyostotic disease and eventually needed operative treatment. In case of bony deformity, corrective osteotomies and rigid internal fixation were performed in addition to curettage, cryosurgery, and bone grafting. In polyostotic disease, expected outcomes were good, but in McCune-Albright syndrome, results were uniformly poor. J. Surg. Oncol. 2001;76:157-166.

Adolescent↗