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PubMedDate not supplied

Fibrous dysplasia.

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Adult↗
PubMed2005

Bisphosphonate treatment of pediatric bone disease.

The science of measuring bone mineral density has developed rapidly and, with it, an improved understanding of the efficacy and safety of various therapeutic interventions in adults. In contrast, the meaning and precision of such measurements in children are equivocal, and the concept of treatment for low bone density in the young patient is still largely undecided. In this report we review the present state of knowledge regarding the use of bisphosphonates during childhood to ameliorate the skeletal abnormalities associated with osteogenesis imperfecta, idiopathic juvenile osteoporosis, fibrous dysplasia of bone and cerebral palsy. Because of the paucity of long-term studies among children regarding the safety and efficacy of these drugs, it is difficult to formulate strong evidence-based recommendations for their use, except perhaps in children with osteogenesis imperfecta.

Bone Density↗
PubMedDate not supplied

[Fibrous osteodysplasia in forensic medicine traumatology].

Histo- and ultrastructure of pathologically changed bone tissue in fibrous dysplasia was under study (9 cases were analyzed). Histologically a fibrous dysplasia focus is represented by cellular fibrous osteogenic tissue with scanty primitive coarse-fibrous bone structures. Ultrastructural changes evidence that these structures are formed at the edge of previous bone beams. Inadequate intraperiod fine structure of collagen fibrils does not provide a strong connection between hydroxyapatite crystals. These shifts result in functional inadequacy of bone tissue in fibrous dysplasia, that should be taken into consideration when assessing the severity of injury to osseous organs.

Adolescent↗
PubMed2001

Detection of c-fos expression in benign and malignant musculoskeletal lesions.

The proto-oncogene c-fos has been implicated in the development of both benign and malignant lesions of bone. Although c-fos expression in such lesions has been well studied in transgenic mouse models, less is known about its role in human musculoskeletal pathology. To clarify this relationship, we used in situ hybridization to localize c-fos m-RNA transcripts in 26 fibrous lesions (eight cases of extra-abdominal fibromatosis and six cases each of fibrous dysplasia, fibrosarcoma, and malignant fibrous histiocytoma of bone) as well as six chondrosarcomas and eight conventional high grade osteosarcomas. We found detectable levels of c-fos expression in tissues from each type of lesion tested. Moreover, all fibrous lesions consistently demonstrated high levels of expression in a majority of cells in each lesion. Chondrosarcomas and osteosarcomas exhibited more heterogeneity in c-fos expression than fibrous tissues. Three of six chondrosarcomas showed moderate expression of c-fos while only one of six was considered high. Similarly, only three of eight osteosarcomas had high expression of c-fos. These findings indicate that the expression of c-fos may be important in the development of a broad range of fibrous lesions as well as in bone and cartilaginous tumors. Additionally, this is the first report, to our knowledge, of detectable c-fos m-RNA in human chondrosarcoma.

Adolescent↗
PubMed2003

Fibrous dysplasia in the maxilla: possible mechanism of bone remodeling by calcitonin treatment.

The long-term follow-up case of monostotic fibrous dysplasia of the maxilla in a 10-year-old girl is described with her endocrinologic data and therapeutic consequence of calcitonin administration in association with surgical interventions. The fibrous dysplasia tends to become more quiescent or static after skeletal growth ceases, but the causative has been still unknown to date. In this case reported changes of calciotropic hormones in the serum were well corresponding to the ceasing of the tumor growth at the puberty and reflected to the calcitonin administration. Although calcitonin has been applied to the fibrous dysplasia associated with McCune Albright syndrome, no histologic changes have been described after the calcitonin treatment. This report might be the first description of bone remodeling after the calcitonin treatment for the fibrous dysplasia of the maxilla in immature people. Data of this case may provide a clue to the pathogenesis of fibrous dysplasia. Surgical intervention can be performed after the local bone calcification by a calcitonin treatment, because of alleviation of vigorous hemorrhage by the bone remodeling.

Bone Remodeling↗
PubMed1998

The "Taiwanese giant": hormonal and genetic influences in fibrous dysplasia.

A 14-year-old boy has been followed for 4 years with a rapidly growing, recurrent area of fibrous dysplasia of the left maxilla and zygoma following resection and bone grafting. Standing 190 cm tall, he was found to have elevated serum growth hormone levels and a pituitary adenoma. His case appears to represent a postzygotic gene mutation of McCune-Albright syndrome. It is possible that the elevated growth hormone levels are in part responsible for the rapid progression of the tumor.

Adolescent↗
PubMedDate not supplied

Fibrous dysplasia of the ethmoid sinus.

Fibrous dysplasia of bones comprising the paranasal sinuses is well recognized. Depending on the anatomic areas of the skull involved by this neoplasm, a combined approach for surgical removal may be necessitated. A case of a patient with fibrous dysplasia of the ethmoid sinus, possibly the first reported case of such change in the ethmoid bone itself, is presented. A two-stage, combined otolaryngologicneurosurgical craniofacial resection was the method of treatment.

Adult↗
PubMed2003

Fracture incidence in polyostotic fibrous dysplasia and the McCune-Albright syndrome.

UNLABELLED: In patients with polyostotic fibrous dysplasia of bone, the peak incidence of fractures is during the first decade of life, followed by a decrease thereafter. Phosphaturia is associated with an earlier incidence and increased frequency of fractures. INTRODUCTION: Fibrous dysplasia (FD) is a disorder involving either one (monostotic) or several bones (polyostotic FD [PFD] and sometimes is associated with cafe-au-lait hyperpigmentation of the skin and one or more hyperfunctioning endocrinopathies (McCune-Albright syndrome [MAS]). Both PFD and MAS are often associated with phosphaturia. Although fractures occur frequently in PFD/MAS, fracture incidence and the effect of age and co-existing metabolic abnormalities (endocrinopathy and/or phosphaturia) on fractures are ill defined. MATERIALS AND METHODS: We reviewed the medical records and examined the endocrine and phosphorus metabolism of 35 patients with PFD/MAS. We report on the age at which extremity fractures occurred and their location and treatment. The results of endocrine and phosphorus metabolism testing and associations between age of first fractures, number of fractures, fracture rate, and metabolic abnormalities were noted. RESULTS: The average follow-up was 14.2 years (range, 2-39 years), during which 172 fractures occurred. The number and sites of fractures were 103 femoral, 25 tibial, 33 humeral, and 11 forearm. Twenty-seven patients had PFD with one or more endocrinopathies and/or phosphaturia, and eight had PFD alone. The endocrinopathies included precocious puberty (n = 19), hyperthyroidism (n = 9), growth hormone excess (n = 6), and one patient each with Cushing syndrome and primary hyperparathyroidism. Twelve patients had phosphaturia. The peak rate of fractures occurred between 6 and 10 years of age and decreased thereafter. Patients with metabolic abnormalities sustained their first fracture at an earlier age (6.9 versus 16.6 years, p < 0.005) and had a higher lifetime rate of fractures (0.29 versus 0.08 fractures/year), relative to patients with PFD alone. Phosphaturia was the single metabolic dysfunction associated with both an earlier age of first fracture (5.1 versus 16.6 years, p < 0.05) and a greater lifetime fracture rate (0.35 versus 0.08 fractures/year, p < 0.05). CONCLUSIONS: The occurrence of extremity fractures in FD peaks between 6 and 10 years of age and declines thereafter. Fractures occur earlier and more frequently in the presence of phosphaturia. These data have implications for long-term prognosis, clinical management, and interpretation of therapeutic interventions.

Adolescent↗
PubMed2002

Lesions related to the formation of bone, cartilage or cementum arising in the oral area: a statistical study and review of the literature.

This report contains a statistical review of 559 cases of lesions forming hard tissues that were diagnosed by the departments of Clinical Pathophysiology and of Pathology at Tokyo Dental College from 1966 to 2001. Sixteen kinds of lesions which were related to the formation of bone, cartilage or cementum were analysed: osteoma, osteo-chondroma, chondroma, osteoid osteoma, osteoblastoma, ossifying fibroma, cemento-ossifying fibroma, cementifying fibroma, so-called cementoma, cementoblastoma, gigantiform cementoma, periapical cemental dysplasia, osteosarcoma, chondrosarcoma, fibro-osseous lesion, and fibrous dysplasia of bone. The most common lesion was osteoma (203 cases). There is a marked tendency for this condition to occur in females (201 males cases and 358 female cases). The patients' ages ranged from 3 to 84 years, and the mean was 40.1 years old. Lesions with hard tissue formation were observed most frequently in the third decade and in the mandibular molar region.

Adolescent↗
PubMed2001

Establishment and characterization of a cell line from a malignant fibrous histiocytoma of bone developing in a patient with multiple fibrous dysplasia.

PURPOSE AND METHODS: In order to provide material for genetic analysis of fibrous dysplasia (FD), a cell line designated GBS-1 was established from a secondary bone malignant fibrous histiocytoma (MFH) developing in a rib of a 44-year-old male polyostotic FD patient. RESULTS: The GBS-1 cells are characterized by a pleomorphic spindle cell morphology with abundant mucus production. On transplantation to nude mouse subcutis the cell line forms myxoid-spindle cell sarcomas with giant cells, the myxoid product being positive for periodic acid-Schiff (PAS) and alcian blue (Al-B) stains and completely digested by hyaluronidase, mimicking the original tumor. Chromosome and genetic analyses revealed multiple structural and numerical abnormalities of chromosomes with a large number of unidentifiable chromosomes and p53 mutation in exon 7 with LOH in the counterpart. CONCLUSIONS: Since cell lines for FD have hitherto not been available, the GBS-1 cells should prove useful for genetic analyses of FD and also MFH of bone origin.

Adult↗
PubMed2006

Adamantinoma of tibia: a study of 12 cases.

BACKGROUND: Adamantinoma is a rare tumor of long bones that can be mistaken for a carcinoma, and numerous other lesions such as myoepithelial tumor, malignant mixed tumor of bone, fibrous dysplasia/osteofibrous dysplasia, and vascular tumors. METHODS: The histology and clinical features of 12 cases accrued from the surgical pathology files over a period of 23 years were analyzed. RESULTS: Of the 12 cases, 4 were men and 8 women, with a mean age of 30 years. Radiologically the tumor presented as a lytic, eccentric, intracortical lesion in the tibia. The histology revealed epithelial islands with basaloid, spindle, and squamous features on a background of fibrotic stroma. Three patients came back with local recurrences, and one of them developed pulmonary metastasis. DISCUSSION: In this article, an attempt is made to study the morphology, biology, and the differential diagnoses of adamantinoma of long bones.

Adamantinoma↗
PubMedDate not supplied

Ultrastructure of low-grade intraosseous osteosarcoma of bone: a comparative study with fibrous dysplasia and parosteal osteosarcoma.

The ultrastructure of low-grade intraosseous osteosarcoma (LGOS) is not well documented in the literature. Four cases of LGOS are described with an emphasis on its distinguishing characteristics as well those it shares with other lesions. The predominant cells of LGOS are fibroblasts with well-developed rough endoplasmic reticulum and mild focal immunoreactivity to SMA and MSA. Few osteoblasts and myofibroblasts are present. Transition cells between fibroblasts and osteoblasts are also noted. The fibroblasts are closely related to osteoblasts from a histological and functional point of view, and phenotypically are probably modified osteoblasts. Comparative ultrastructural studies between LGOS and fibrous dysplasia (FD) reveal basic similarities, although the cells in LGOS are larger with more of an abundance of organelles. Therefore, accurate differentiation between these two lesions rests at the histological and radiological levels. LGOS and parosteal osteosarcomas (PO) also share similar ultrastructural features. In the case samples in this study, an unusual type of multilayered amorphous material was found in the osteoid matrix of a case of LGOS and one of PO. This probably emphasizes the morphologic similarities between these 2 tumors.

Adult↗