SEARCH · PubMed Health
Results for “FIBROUS DYSPLASIA OF BONE”
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to expressions of c-fos and c-jun products and bone matrix proteins: a clinicopathologic review and immunohistochemical study of c-fos, c-jun, type I collagen, osteonectin, osteopontin, and osteocalcin.
Fibrous dysplasia and osteofibrous dysplasia are both benign fibro-osseous lesions of the bone and are generally seen during childhood or adolescence. Histologically, the features of these bone lesions sometimes look quite similar, but their precise nature remains controversial. We retrospectively studied clinicopathologic findings in 62 cases of fibrous dysplasia and 20 cases of osteofibrous dysplasia with regard to their anatomic location and histological appearance. From among these cases, the immunohistochemical expressions of c-fos and c-jun proto-oncogene products and bone matrix proteins of type I collagen, osteonectin, osteopontin, and osteocalcin were evaluated in 20 typical fibrous dysplasias and 17 osteofibrous dysplasias using paraffin sections, and these expressions were then assessed semiquantitatively. Microscopically, fibrous dysplasia showed various secondary changes, such as hyalinization, hemorrhage, xanthomatous reaction, and cystic change in 22 of the 62 cases (35%). This was a higher incidence than in osteofibrous dysplasia, in which only 2 of the 20 cases (10%) showed such changes. In the elderly fibrous dysplasia cases, the cellularity of fibroblast-like cells was rather low, and those cases were hyalinized. Almost all of the cases of fibrous dysplasia and osteofibrous dysplasia showed positive expressions of c-fos and c-jun products. The expressions of type I collagen and osteopontin showed no difference between fibrous dysplasia and osteofibrous dysplasia. Immunoreactivity for osteonectin in bone matrix was detected in only 1 case of fibrous dysplasia (1 of 20), whereas it was recognized in 14 of the 17 cases of osteofibrous dysplasia. Furthermore, the immunoreactivity for osteocalcin in bone matrix and fibroblast-like cells was higher in fibrous dysplasia than it was in osteofibrous dysplasia, semiquantitatively. Our immunohistochemical results regarding osteonectin and osteocalcin suggest that the bone matrix of fibrous dysplasia is somewhat more mature than that of osteofibrous dysplasia, and that the fibroblast-like cells in fibrous dysplasia share some phenotypic features with osteoprogenitor cells of normal osteogenic tissues. Fibrous dysplasia and osteofibrous dysplasia share some similar histological features, including c-fos and c-jun expressions, although different clinicohistologic features and immunohistochemical expressions of osteonectin and osteocalcin were observed. These features suggest that the mechanisms behind the development of fibrous dysplasia and osteofibrous dysplasia are similar, but this is not necessarily indicative of a closer relationship between the 2 diseases.
[Pathological fracture in osteofibrous dysplasia].
Osteofibrous dysplasia Campanacci is a rare tumor-like lesion mostly presented in the meta-epiphyseal region of the tibia. Different options exist about the treatment modalities. The rare case of a 4 1/2-year-old girl suffering from a pathologic fracture of the tibia shaft due to a osteofibrous dysplasia is presented. The fracture was stabilized by intramedullary nailing using elastic titanium rods. During this procedure a biopsy of the lesion confirmed the radiological suspected diagnosis of an osteofibrous dysplasia. The implant was removed after ten months when fracture and lesion were healed. The treatment options and the differential diagnoses are discussed by review of the literature.
An unusual case of mixed sclerosing bone dystrophy presenting with morning stiffness and joint swelling in childhood: a case report.
We describe a case of a 16 year old native Indian girl with pain, morning stiffness and foot swelling who had radiographic changes consistent with mixed sclerosing bone dystrophy (MSBD) combined with fibrous dysplasia.
Occipital fibrous dysplasia tonsillar herniation and cervical syringomyelia.
A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foraman magnum has been implicated in the development and subsequent progression of syringomyelia. This report highlights the gradual development of a small posterior fossa due to fibrous dysplasia of the occipital bone with consequent tonsillar herniation resulting in syringomyelia.
[Circumscribed form of fibrous degeneration of the cranial bones].
Explore the source record for details and available documents.
Fibrous dysplasia associated with sickle-cell disease. Report of a case with a discussion of the multiple factors affecting both conditions.
Explore the source record for details and available documents.
[Fibrous dysplasia: differential diagnosis from Paget's disease].
HISTORY AND CLINICAL FINDINGS: A 27-year-old woman presented with chronic diffuse bone pain and skeletal deformities. Since the age of 3 years she had occipital hyperostosis. Since aged 13 years she had symptoms indicating spinal root involvement due to hyperkyphosis. For the last 6 years there was evidence of destructive and hyperplastic changes in the region of the middle ribs. Alkaline phosphatase (APL) had been elevated (> 250 U/l) for several years. Three years before the present admission the patient had been hospitalized elsewhere because of bone pain and had received intermittent infusions of pamindronate to a total of 600 mg. INVESTIGATIONS: Laboratory tests showed moderate rise in bone-specific APL and osteocalcin. Conventional x-ray examinations and computed tomography showed lesions in the occipital bone, sella turcica and ethmoid air cells. Several thoracic vertebral fractures were demonstrated. Skeletal scintigraphy indicated enrichment in the occipital and supraorbital regions, as well as in vertebrae and ribs. Dual energy x-ray absorptiometry (DEXA) showed osteopenia. Bone biopsy from the occipital lesion revealed fibrous dysplasia. TREATMENT AND COURSE: The diagnosis of fibrous dysplasia having been confirmed, cyclical treatment with i.v. pamidronate was given for 6 months. It was well tolerated and lessened the pain without changing the objective findings. CONCLUSION: In case of uncertain radiological and scintigraphic findings in the differential diagnosis of fibrous dysplasia, Paget's disease or bone tumor, it is essential to obtain a biopsy and, if available, perform gene analysis.
Osteofibrous dysplasia-like adamantinoma of bone: a report of five cases with immunohistochemical and ultrastructural studies.
This report describes five cases of osteofibrous dysplasia-like adamantinoma of the tibia in young patients ranging from ages 4 1/2 to 14 years. Radiologically and histologically, these cases were indistinguishable from osteofibrous dysplasia of bone, and no epithelial cells were recognized on routine staining. However, epithelial differentiation was seen in the form of scattered keratin-positive cells in all five cases, and tonofilaments in four cases. The patients were treated by curettage, and three had recurrences. Follow-up showed no progression to classic adamantinoma. Osteofibrous dysplasia-like adamantinoma is a special histological type of adamantinoma that affects children and adolescents. It differs from classic adamantinoma in that it lacks conspicuous nests and masses of epithelial cells, and the prognosis after conservative treatment is generally good. Recent publications suggest that osteofibrous dysplasia-like adamantinoma is a precursor of classic adamantinoma. In a comparative study of three cases of classic adamantinoma, we found, in the fibroblastic stroma of the tumors, spindle epithelial cells that were indistinguishable from the epithelial cells of osteofibrous dysplasia-like adamantinoma. This finding suggests that there is an overlap between these conditions. Four additional cases of osteofibrous dysplasia of the tibia from our files lacked epithelial differentiation. It is most likely that osteofibrous dysplasia is part of the morphologic spectrum of adamantinoma.
Pathological fractures in osteopathies and bony dysplasias.
Explore the source record for details and available documents.
Radiological difficulties in the diagnosis of fibrous dysplasia of the sphenoid sinus and the cranial base.
Fibrous dysplasia is a benign disorder of bone. We present the case of a 26-year-old man with fibrous dysplasia of the sphenoid sinus and the cranial base. The presentation and management of this rare condition are discussed highlighting the potential for misdiagnosis with MRI.
[ON THE ROENTGENOLOGICAL DIFFERENTIAL DIAGNOSIS OF SOME TUMORAL AND TUMOR-LIKE DISEASES OF THE MANDIBLE].
Explore the source record for details and available documents.
Classic adamantinoma with osteofibrous dysplasia-like foci and secondary aneurysmal bone cyst.
Adamantinoma, a rare bone lesion of the tibia and fibula, has two distinct variants, classic adamantinoma and osteofibrous dysplasia-like adamantinoma. Composite lesions have not been described. Aneurysmal bone cyst is a benign cystic lesion which may also occur in the tibia and fibula. We report an unusual case of classic adamantinoma with osteofibrous dysplasia-like areas and foci of secondary aneurysmal bone cyst with prominent giant cells. A lesion was diagnosed in a 17-year-old girl with a 14-year history of a slowly enlarging left tibial mass and increasing deformity. Pathologically, the predominant pattern was classic adamantinoma, with minor foci of osteofibrous dysplasia-like adamantinoma and areas of secondary aneurysmal bone cyst with abundant multinucleated giant cells. We report the clinical, radiologic, and pathologic features of this case, and summarize lesions associated with secondary aneurysmal bone cyst. To our knowledge, the association of adamantinoma with secondary aneurysmal bone cyst has not been previously reported.
[Problems in treating the "aggressive" form of fibrous dysplasia (author's transl)].
Explore the source record for details and available documents.
Pathological fractures in benign tumours, pseudotumours, and localised dysplasia,.
Explore the source record for details and available documents.
Frontal sinus mucocele: a rare complication of craniofacial fibrous dysplasia.
We present plain radiographic, computed tomographic and magnetic resonance imaging (MRI) findings in a 25-year-old female patient with craniofacial fibrous dysplasia (FD). Although FD has a tendency to involve craniofacial bones in a unilateral fashion, the involvement was bilateral and extensive in this case. An additional feature was the presence of a frontal sinus mucocele, presumably due to the involvement of the sinus recess by the dysplastic process. This complication of the craniofacial FD has been reported very infrequently in the literature.
Malignant transformation of polyostotic fibrous dysplasia.
We have described an unusual case of polyostotic fibrous dysplasia in a 25-year-old white woman who had malignant transformation into a mesenchymal tumor with widespread metastases and features of rhabdomyosarcoma. Masculinization also occurred with high plasma testosterone levels. A mechanism for the development of masculinizing features is discussed.