1/224. Osteofibrous dysplasia of long bones a new clinical entity.A new clinico-pathologic entity is described. It is defined as osteofibrous dysplasia of long bones, and is based on twenty two personal observations to which are added seventeen cases from the literature. This dysplasic congenital lesion is clearly differentiated from fibrous dysplasia by clinical, radiographic and histological characteristics and by its clinical course. These features may be summarised as follows: 1) Slight predominance of the male sex. 2) Very early age of onset either at birth or in the first years of life. 3) Site almost exclusively tibial, sometimes also in the fibula. Localisation predominantly in the middle third of the tibial diaphysis, but sometimes in the distal or proximal third. In the fibula, it is always at the distal third. 4) The lesion is painless and generally causes bony enlargement. There is often slight anterior bowling and more rarely, slight varus of valgus bowing. Pathological fracture may occur; rarely there is a pseudarthrosis. 5) The radiographic appearances are very characteristic, with enlargement of the bone, intracortical osteolytic lesions with thinning or disappearance of the external cortex, sclerotic reaction on the medullary aspect, and narrowing of the medullary canal. 6) The histological features are also typical, consisting of fibrous tissue enclosing bone trabeculae lined by osteoblasts and a "zonal" architectural pattern. 7) Sometimes the lesion tends to heal spontaneously in the very early years of life; in other cases it is moderatley progressive. It relapses frequently after curettage, but such recurrences are generally non-progressive. In some cases slight anterior bowing persists permanently. 8) Surgery should be restricted to patients over the age of five in whom the lesion is extensive, with imminent or actual pathological fracture, and to the rare cases of pseudarthrosis. The results are good even in cases of relapse or pseudarthrosis. The correction of residual bowing, if indicated, can safely be carried out with one or more osteotomies at the age of ten to twelve years.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/224. Cushing's syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome.McCune-Albright syndrome consists of fibrous dysplasia of bone, cafe-au-lait skin pigmentation, and endocrine dysfunction (usually precocious puberty). Other endocrine abnormalities occur in a minority of patients, and of these, Cushing's syndrome is the least often recognized. We present 5 children (4 girls) with features of McCune-Albright syndrome who had Cushing's syndrome in the infantile period (<6 months). In 2 children spontaneous resolution occurred, but the remaining 3 required bilateral adrenalectomy. In addition, all 4 girls have experienced precocious puberty, and 3 children demonstrated radiologic evidence of nephrocalcinosis. Understanding of the underlying defect causing McCune-Albright syndrome emphasizes the importance of searching for other endocrine dysfunction in these children.- - - - - - - - - - ranking = 0.14893116965613keywords = bone, endocrine (Clic here for more details about this article) |
3/224. Malignant spindle cell tumor arising in the mandible of a patient with florid osseous dysplasia.Florid osseous dysplasia is a non-neoplastic condition of the alveolar processes of the jaws characterized by the replacement of multiple foci of bone by fibrous connective tissue, accompanied by gradual deposition of cementum, bone, or both. The lesions are not associated with inflammatory diseases of the dental pulp or periodontal tissues. In fully developed florid osseous dysplasia, there are multiple lobulated masses in the alveolar bone bilaterally in the mandible and sometimes in the maxilla. This is the first report of a malignancy originating within the jaws of a patient with florid osseous dysplasia. A spindle cell malignancy was diagnosed in the mandible of a 54-year-old black woman whose jaw was affected by florid osseous dysplasia bilaterally. Despite extensive surgery and radiotherapy, the patient died 20 months after diagnosis of the malignancy.- - - - - - - - - - ranking = 0.42857142857143keywords = bone (Clic here for more details about this article) |
4/224. Giant central ossifying fibroma of the mandible: report of case.A case is presented of a patient with a giant ossifying fibroma of the mandible that had been diagnosed earlier as fibrous dysplasia. Surgical resection of the tumor and involved bone was chosen as the mode of treatment because of the extent of the lesion and continued infection. Surgeons should be aware that ossifying fibroma is a very distinct possibility in large growing lesions in age groups older than is deemed compatible with fibrous dysplasia.- - - - - - - - - - ranking = 0.14285714285714keywords = bone (Clic here for more details about this article) |
5/224. Two cases of secondary angiosarcoma arising from fibrous dysplasia.Angiosarcoma associated with fibrous dysplasia is very rare. We have recently experienced two cases of angiosarcoma that secondarily arose from fibrous dysplasia. The first patient, a 55 year-old man, had noticed a deformity in the right upper arm since he was five years old. At the age of 25 years, polyostotic fibrous dysplasia was diagnosed by X-ray examination. The patient complained of swelling and pain around the left shoulder. The diaphysis of the humerus was mostly non-observable due to severe bone destruction by tumor invasion and there was a large soft tissue tumor. biopsy examination revealed grade II or III hemangioendothelioma with typical histologic findings of fibrous dysplasia. After interscapulothoracic amputation for wide tumor resection, he died of DIC. autopsy revealed multiple liver metastatic lesions of angiosarcoma. The second patient was a 66-year-old woman. She presented with a pathological fracture in the right tibia, due to an osteolytic lesion with cortical bone swelling and thinning. The histologic diagnosis was fibrous dysplasia without any sarcomatous changes. However, after a 3-time recurrence, angiosarcoma arose from the same lesion. She is now still alive with disease-free period of 6.5 years after amputation.- - - - - - - - - - ranking = 0.28571428571429keywords = bone (Clic here for more details about this article) |
6/224. Cystic fibrous dysplasia mimicking giant cell tumor: MRI appearance.We report the case of a 43-year-old man who presented with an osteolytic and expansive lesion in the left distal femur mimicking a giant cell tumor. Magnetic resonance imaging (MRI) showed that most of the lesion was cystic, and histological examination revealed fibrous dysplasia with marked cystic degeneration. Radiographic findings of cystic fibrous dysplasia in the end of a long bone may be similar to those of a giant cell tumor, and a biopsy is essential for the final diagnosis.- - - - - - - - - - ranking = 0.14285714285714keywords = bone (Clic here for more details about this article) |
7/224. Fibrous dysplasia of the temporal bone: report of a case and a review of its characteristics.Fibrous dysplasia is an uncommon benign disorder of unknown etiology. It represents a disturbance of normal bone development--specifically a defect in osteoblastic differentiation and maturation that originates in the mesenchymal precursor of the bone. Because fibrous dysplasia shows a predilection for the facial and cranial bones, where it causes deformity and dysfunction, the disease is of particular interest to the otolaryngologist. In this paper, we report a case of fibrous dysplasia of the temporal bone, the first symptom of which was a mixed hearing loss. We discuss the characteristic features of this specific location of the disease, the differential diagnosis, and the treatment policy. We also address the issue of secondary sensorineural hearing loss.- - - - - - - - - - ranking = 1.1428571428571keywords = bone (Clic here for more details about this article) |
8/224. Fibrous dysplasia arising from the calcaneus.A case of an 18-year-old woman with fibrous dysplasia arising in the calcaneus, which is extremely rare, is reported, with the emphasis placed on differential diagnosis from low-grade central osteosarcoma. She had a severe pain in her left ankle after sprain. Plain radiographs showed a radiolucent lesion measuring 6.3 x 2.5 cm with a sclerotic margin in the left calcaneus. CT scans showed a well-defined lytic lesion with disruption of the lateral cortex and an ossification or calcification in its center. On MR imaging, the lesion had isointensities and high intensities on T1 and T2 weighted images, respectively, but its central portions showed lower intensities both on T1 and T2 weighted images. The lesion was enhanced with gadolinium except for the central portions. The specimen obtained by open biopsy consisted of fibrous tissue and foci of irregular woven bone. None of the nuclear atypia, mitoses, longitudinal stream of bone or invasive nature of growth was detected. The diagnosis of fibrous dysplasia was histologically made. The lesion was curetted and packed with autogenous bone chips. No evidence of recurrence was noted postoperatively.- - - - - - - - - - ranking = 0.42857142857143keywords = bone (Clic here for more details about this article) |
9/224. Repeated dedifferentiation of low-grade intraosseous osteosarcoma.Low-grade intraosseous osteosarcoma is an uncommon bone tumor that is characterized by minimum cytological atypism and a much better prognosis than conventional osteosarcoma. This report describes a patient who had a low-grade osteosarcoma that mimicked fibrous dysplasia (FD). The tumor had an area of high-grade sarcoma at the initial diagnosis. Ten years after incomplete resection of FD-like tumor, local recurrence with areas of high-grade tumor developed. This case illustrates the potential of dedifferentiation in low-grade intraosseous osteosarcoma.- - - - - - - - - - ranking = 0.14285714285714keywords = bone (Clic here for more details about this article) |
10/224. Juvenile ossifying fibroma of the mandible. An 8 year radiological follow-up.An 8-year follow-up of a juvenile ossifying fibroma in the left mandible of a 4-year-old boy is presented. The case demonstrated initial lack of radiological evidence of demarcation and subsequent eccentric enlargement, selective tooth displacement and a multilocular appearance in areas of active growth. An aneurysmal bone cyst and a decrease in the bone content was present in the excision specimen.- - - - - - - - - - ranking = 0.28571428571429keywords = bone (Clic here for more details about this article) |
| | Next -> |