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At least 811 records · Page 45Linked to original sources
PubMed2003

Fibrous dysplasia of the ethmoid sinus.

Although craniofacial bone is the second common site of fibrous dysplasia involvement, it is rarely found in the paranasal sinus. Among fibrous dysplasia of the head and neck, the maxilla and mandible are the most frequent sites to be involved. Fibrous dysplasia becomes dormant in adolescence and early adult life and is more common in female. It is one of the fibrous osseous lesions and should be differentiated from osteoma and ossifying fibroma. Radiographically, fibrous dysplasia showed "groundglass" bone appearance on CT scans with bone window. Histopathologically, it presents woven-type bone embedded in a cellular fibrous stroma without osteoblastic rimming. We presented a case of 25-year-old female with fibrous dysplasia in her right side ethmoid sinus. She visited to us with the chief complaint of right side headache since adolescence. The lesion was removed by endoscopic sinus surgery and pathology proved fibrous dysplasia. The patient was free of headache after operation. The advance of endoscopic sinus surgical technique, makes it an optimal method for the pathological diagnosis and treatment to avoid the cosmetic problems caused by external approach in limited paranasal sinus fibrous osseous lesions.

Adult↗
PubMed2004

Pathologic hepatic Tc-99m-MDP uptake in polyostotic fibrous dysplasia.

Fibrous dysplasia of bone is a congenital, sporadic developmental disorder characterized by immature fibrous connective tissue and bone deformities. Hepatic Tc-99m-MDP uptake is a rare, serendipitous finding during bone scanning studies. The present patient was a 25-year-old male who had severe polyostotic fibrous dysplasia. On Tc-99m-MDP (methylene diphosphonate) bone scintigraphy, increased activity accumulations were seen on multiple ribs, vertebrae and base of the cranium. In addition, diffuse increased pathologic uptake of Tc-99m-MDP in the liver was shown. Intravenous pamidronate was administered monthly for two months. In the third week of the last administration Tc-99m-MDP bone scintigraphy was performed again, but despite sustained bone involvement, pathologic hepatic uptake was not seen on the scan. We thought that pathologic hepatic Tc-99m-MDP accumulation, may be related to the formation and aggregation of calcium oxalate and phosphate crystals which improved with pamidromat treatment.

Adult↗
PubMedDate not supplied

The treatment of monostotic fibrous dysplasia of the first metatarsal with free vascularized fibular bone graft.

Historically, patients suffering from fibrous dysplasia of the metatarsals faced amputation or resection as the treatments of choice. Although the lesion itself is not malignant, pain, swelling, and disability dictated the treatment. With recent refinements in microvascular surgery, vascularized bone grafting has offered a viable alternative for this and similar conditions that offers the potential for a functional, pain-free foot.

Adult↗
PubMedDate not supplied

CT of the "Tegernsee Giant": juvenile gigantism and polyostotic fibrous dysplasia.

We report the radiological findings in the unusual case of the Bavarian "Tegernsee Giant." With conventional radiography, CT, and histologic examination, we succeeded in diagnosing two disorders: The Tegernsee Giant suffered from (a) juvenile gigantism caused by a growth hormone-secreting tumor of the pituitary gland and (b) a polyostotic form of fibrous dysplasia of the skull and multiple bones particularly on the left side of the body.

Adult↗
PubMed1977

[Fibrous dysplasia].

Fibrous dysplasia of bone may occur also in the facial skeleton. Main locations are the lateral midface and the mandible. The foci, which are sharply circumscribed in most cases, produce tumour-simulating deformities of single of several facial bones and, in part, disorders of the articulation are bulbar displacements. In contrast to Paget's disease, fibrous dysplasia is a disorder of the younger age groups. Apart from bony changes, Albright's syndrome (which occurs in women only) comprises also irregular, circumscribed café-au-lait patches and signs of somatic and sexual precocity.

Facial Bones↗
PubMed2002

Melatonin deficiency and fibrous dysplasia: might a relation exist?

Fibrous dysplasia of bone might be monostotic, polystotic, or occurs as a part of McCune-Albright syndrome and Jaffe-Lichtenstein syndrome. Activating mutations of GNAS1 gene was identified in patients with fibrous dysplasia. However, fibrous dysplasia might occur in the absence of these mutations and fibrous dysplastic tissue was produced in vitro by the effects of excess exogenous cAMP on human osteogenic cells. It was proved that the fibrous dysplastic tissue is deficient in bone sialoprotein. Melatonin deficiency might be hypothesized in syndromes associated with fibrous dysplasia or formation of fibrous dysplasia-like tissue. The receptor RZR/ROR is the nuclear receptor of melatonin and the human bone sialoprotein gene contains a RZR/ROR response element. It was supposed that binding of melatonin to its membrane receptors results in changes in the levels of activity of nuclear cAMP that lead to alteration of expression of bone sialoprotein. Also, melatonin deficiency might increase cAMP in bone through its effect on prostaglandins of the E group. Further, melatonin deficiency might explain precocious puberty in cases of McCune-Albright syndrome. We might hypothesize that melatonin deficiency might play a role in development of fibrous dysplasia in some cases.

Bone Development↗
PubMedDate not supplied

Condylocephalic nailing for pathologic subtrochanteric fracture.

Pathologic subtrochanteric fracture in a femur rarefied by fibrous dysplasia of bone was successfully treated with condylocephalic nailing and bone grafting. This procedure is recommended not only for traumatic fractures but also for pathologic fractures secondary to bone tumor.

Adolescent↗
PubMed2003

Paget's disease and fibrous dysplasia.

Paget's disease and fibrous dysplasia are benign disorders that can involve the temporal bone and skull base. They commonly lead to otolaryngologic symptoms such as impingement of cranial nerves or the orbit or blockage of the external auditory canal or paranasal sinuses, although they can often be a challenge to diagnose because of their insidious presentation. Their benign nature and common presentation within the difficult-to-access confines of the skull base should lead the clinician to exercise caution in their treatment, reserving surgical intervention for either diagnosis or the relief of symptoms. As a better understanding of the etiology of these conditions develops and new pharmacotherapeutic agents are tested, it is likely that physicians will be able to turn to medical rather than surgical techniques to treat these disorders.

Ethmoid Bone↗
PubMedDate not supplied

Fibrous dysplasia.

The controversy is this case centers around the management of this lesion, not the differential diagnosis. All the consultants agreed that the history, physical, and CT findings were consistent with various fibroosseous lesions, the most likely being fibrous dysplasia. The need for additional tests varied with a bone scan (Dr. Kearns), a bone scan and CT scan (Dr. McGill), and MRI, MRI angiogram, bone survey, BUN, creatinine, calcium, and phosphorus (Dr. Potsic). Cosmetic and functional changes were considered priorities for the consultants, with orbital compression, malocclusion, tooth eruption, nasal obstruction, and sinusitis (Dr. Kearns), nasolacrimal duct obstruction and orbital compression (Drs. McGill and Potsic) being the concerns. Because this lesion is benign and slow-growing, the consensus is that surgery should be reserved for functional or cosmetic compromise. But how aggressive should one be and what approach should be used? The approaches varied with midface degloving or lateral rhinotomy (Dr. Kearns), midface degloving (Dr. McGill), or a Caldwell-luc and lateral rhinotomy (Dr. Potsic). Assessment of this tumor postoperatively should be with patient examinations and serial CT scans. None of the consultants worried about sarcomatous changes in this tumor.

Facial Bones↗
PubMed1990

The relationship between bone morphogenetic protein and neoplastic bone diseases.

The monoclonal antibody against bovine bone morphogenetic protein was used for demonstration of bone morphogenetic protein (BMP) in neoplastic bone diseases. The avidin-biotin-peroxidase complex method demonstrated that BMP mainly exists in the cytoplasm of tumor cells of osteosarcoma and chondrosarcoma. Immunostaining showed that a majority of osteosarcomas and all of the chondrosarcoma cells contained a large quantity of BMP. Conversely, none of the fibrosarcomas showed positive staining. Thus, it was possible to differentiate osteosarcomas from fibrosarcomas by immunostaining. In fibrous dysplasia of bone, BMP was abundant in the fibrocellular tissue that had osteogenic activity. In contrast, fibrous tissue of ossifying fibroma showed weak positive staining; only the osteoblasts rimming the bone showed a positive reaction. Immunostaining showed that BMP was also detected in other neoplastic bone diseases such as osteoma, chondroma, and other tumors.

Antibodies, Monoclonal↗
PubMed1994

Unusual manifestations of craniofacial fibrous dysplasia: clinical, endocrinological and computed tomographic features.

Clinical, endocrinological and computed tomographic features of three patients with unusual manifestations or complications of craniofacial involvement of fibrous dysplasia are presented. One patient with polyostotic fibrous dysplasia presented in late pregnancy with acute onset of bilateral optic nerve compression and blindness secondary to a rapidly expanding mass of fibrous dysplasia tissue involving the sphenoid, pituitary and optic chiasm regions. A second patient with polyostotic fibrous dysplasia developed thyrotoxicosis and probable gigantism/acromegaly in keeping with a rare form of McCune-Albright syndrome. Extensive bony distortion of the skull and facial bones by fibrous dysplasia made clinical recognition of these complications more difficult. A third patient had monostotic fibrous dysplasia with marked sclerosis of the sphenoid bone on plain radiographs which mimicked appearances of a meningioma and resulted in a negative craniotomy as computed tomography was not yet available at the time of presentation. Each case demonstrated rare complications of craniofacial fibrous dysplasia and highlighted the wide spectrum of appearances in which it may manifest, often resulting in overlap and diagnostic confusion with other disease processes. The value of computed tomography in assessment is emphasized.

Adolescent↗
PubMed1998

Increased bone mineral turnover without increased glucose utilization in sclerotic and hyperplastic change in fibrous dysplasia.

Fibrous dysplasia is a benign bone disorder. It is diagnosed by distinctive X-ray radiography, CT, and MRI findings. Although bone scintigraphy helps to identify the tumor origin according to accelerated bone turnover, the glucose metabolism in fibrous dysplasia has not yet been investigated. We reported a case of fibrous dysplasia in craniofacial bone which showed signs of the acceleration of bone mineral turnover without elevated glucose utilization by Technetium-99m-HMDP SPECT and Fluorine-18-FDG PET. We concluded that the growth of fibrous dysplasia needed the acceleration of bone mineral turnover without an increase in glucose metabolism.

Bone Density↗