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[Monostotic fibrous dysplasia of the ribs].
The clinical features, macroscopic and radiologic findings and typical histologic alterations in three cases of fibrous dysplasia of ribs are described. In one case the lesion presented as a tumor-like growth of a size unprecedented in previously reported cases. In differentiating this condition from ossifying fibroma, the pattern of distribution of newly formed bone to fibrous connective tissue is shown to be a valuable criterion. In fibrous dysplasia this ratio is constant for any given area, but varies considerably in ossifying fibroma.
Osteofibrous dysplasia (ossifying fibroma of long bones). A study of 12 cases.
Osteofibrous dysplasia (ossifying fibroma of long bones) is one of the fibro-osseous lesions that affects the tibia and fibula in the first decade of life. A study of 12 patients with this lesion showed the high rate of recurrence after surgical intervention. Most commonly, the lesions are eccentrically located in the diaphysis of the tibia. Pseudarthrosis may develop in cases where the lesion is situated in the distal portion. Roentgenologic and histopathologic features are basically similar to those of monostotic fibrous dysplasia of long bones. However, bony or osteoid trabeculae covered by osteoblasts enable distinction between osteofibrous dysplasia and monostotic fibrous dysplasia, as determined histologically. The current evidence indicates that surgery should not be attempted in patients under 10 years of age.
Fibrous dysplasia of the skull and sinuses.
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Early management of pathological fractures in children.
The management of pathological fractures in children remains controversial. The indications for surgical treatment are unclear and the need for histological diagnosis before or after definitive treatment is not clearly defined. We reviewed retrospectively the records of all patients under the age of 16 years who presented over the past 7 years with a fracture as the first manifestation of bone pathology. There were 23 patients (16 boys and 7 girls) of an average age of 12 years and 2 months (range 4.1-15.8 years). There were nine cases of fracture through a simple bone cyst, five cases of fibrous dysplasia, two giant cell tumours, three aneurysmal bone cysts, one chondroblastoma, and three cases of Ewings sarcoma. After review of our cases we propose a simple algorithm for the safe early management and assessment of paediatric pathological fractures. We recommend that primary fixation of pathological fractures should be avoided until histological diagnosis is obtained. Most lesions should eventually be biopsied. However, if radiographic appearances are reassuringly benign, biopsy can be delayed until conservative fracture management is completed. Definitive treatment of benign lesions with protective intra medullary nailing or curettage and grafting can follow frozen section under the same anaesthetic.
Ductal carcinoma in situ in a 27-year-old woman with McCune-Albright syndrome.
McCune-Albright syndrome (MAS) is a sporadic disorder characterized by the triad of irregularly edged hyperpigmented macules (café au lait spots); a slowly progressive bone disorder, polyostotic fibrous dysplasia, usually involving the base of the skull and the long bones; and luteinizing hormone-releasing hormone (LHRH)-independent precocious puberty. This case is the first report of a 27-year-old woman with ductal carcinoma in situ (DCIS) and Paget's disease of the nipple associated with MAS. The discussion focuses on two endocrine manifestations of this syndrome including precocious puberty and excess growth hormone secretion. In our patient, both her early puberty and pituitary adenoma, in association with MAS, resulted in excess production and secretion of estrogen and growth hormone. Both of these hormones function to stimulate breast growth and development. We hypothesize they are responsible for this patient's DCIS and Paget's disease of the nipple so early in life.
Treatment of pathologic femoral neck fractures with modified Pauwels' osteotomy.
UNLABELLED: Treatment of pathologic femoral neck fractures secondary to extensive lesions of fibrous dysplasia and simple bone cysts has been controversial. We think that modified Pauwels' intertrochanteric osteotomy and osteosynthesis can result in sound healing of the lesion and of the fracture, with no recurrence, low complication rates, and good functional results. Seven patients were treated with a uniform approach consisting of biopsy (Stage I) and osteotomy with osteosynthesis (Stage II). Curettage of the lesion and bone grafting were not done. The average followup was 79.28 months (range, 32-142 months). All of the fractures and osteotomy sites healed in means of 14 weeks (range, 12-16 weeks) and 9.1 weeks (range, 8-10 weeks), respectively. All fibrous dysplasia lesions healed radiologically. Grade IV radiographic healing was seen in both patients with simple bone cysts after 35 and 92 months. Using the Musculoskeletal Tumor Society functional evaluation and Toronto Extremity Salvage Score, all the patients had good to excellent results. Complications such as infection, local recurrence, refracture, femoral neck deformity, osteonecrosis of the femoral head, and growth arrest of capital femoral physis were not seen. In addition to biomechanical advantages, the procedure seems to have had a biologic role in healing of the fracture and of the lesion by initiating osteogenesis to replace the defect with new bone. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series--no, or historical control group). See the Guidelines for Authors for a complete description of levels of evidence.
Fibrous dysplasia of the craniomaxillofacial region: current clinical perspectives.
Fibrous dysplasia is a benign fibro-osseous disease of bone of unknown etiology. Its occurrence in the craniomaxillofacial skeleton is frequent and varies in severity from an asymptomatic monostotic lesion to polyostotic involvement resulting in progressive functional deficit and aesthetic problems. With the advent of refined instrumentation and craniofacial surgical techniques, a more aggressive, non-disabling approach to these benign yet deforming fibro-osseous growths is possible. In some patients, complete excision of the involved bone with graft reconstruction of the resultant defect with primary autogenous bone may be possible. Lifelong continuous ongoing monitoring of the involved region is required throughout the patient's life.
[A case of polyostotic fibrous dysplasia (Albright syndrome) with gynecomastia and hyperestrogenism].
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[Essential fibrous dysplasia: several uniosseous forms of the temporal and superior maxillary bone].
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[A case of facial fibrous dysplasia in brothers].
Fibrous dysplasia is a benign bone tumor. The cranio-facial localization concern 20% of fibrous dysplasia. A congenital etiology is evoked by all the authors. Our observations ask for the real etiology of this affection: Congenital? Familial? The diagnosis is done by anatomo-pathology, and surgery is the treatment for severe cases. Relapses are frequent.
Ethmoid fibrous dysplasia with anterior skull base and intraorbital extension.
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[A further case of fibrous dysplasia of the face with misleading symptomatology].
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Cytokeratin expression and distribution in adamantinoma of the long bones and osteofibrous dysplasia of tibia and fibula. An immunohistochemical study correlated to histogenesis.
Twenty-four cases of adamantinoma and 24 cases of osteofibrous dysplasia of the long bones were studied to evaluate the expression and distribution of cytokeratin (CK) subtypes in relation to histogenesis and differentiation. The immunohistochemical study was performed on tissue fixed in buffered formalin and embedded in paraffin wax utilizing antibodies to vimentin, factor VIII, epithelial membrane antigen and cytokeratins of different molecular weights. In all cases the vimentin antibody marked positively in stroma, endothelium and osteoblasts, while factor VIII expression was confined to endothelial cells. In 71% of adamantinomas, vimentin showed strong immunoreactivity in the tumour cells of nests and tubules. CKAE1/AE3 and CK19 were strongly expressed in all morphological patterns of adamantinoma emphasizing their epithelial origin, while the antibodies to CK8 and CK18 showed a high percentage of negative responses. In osteofibrous dysplasia the epithelial-like component was much smaller than in adamantinoma and was present in scattered islands composed of a few cell positive for CKAE1/AE3 and CK19 and negative for other keratins. These results suggest that these two lesions are of a similar histogenesis.
Focal fibrocartilaginous dysplasia: consideration of healing process.
We report two patients with unilateral tibia vara due to focal fibrocartilaginous dysplasia of the medial aspect of the proximal tibia. It appears that tibia vara, caused by a primary self-limited lesion at the proximal medial metaphysis, is corrected by the response of the proximal tibial physis. This finding would indicate that an infantile growth plate of the proximal tibia has a rather large potential to correct an angular deformity at the adjacent metaphysis.
[Monostotic fibrous dysplasia of the upper maxilla. A case report].
Fibrous dysplasia is a rare bone condition characterized by the replacement of healthy bone by fibrous tissue and different grades of bone metaplasia. It may affect one (monostotic) or more bones (polyostotic). We present the case of a patient with monostotic fibrous dysplasia of the upper maxilla and analyze the clinical characteristics, diagnostic and therapeutic aspects, and the bibliography of the disease.