1/309. Florid cemento-osseous dysplasia. Report of a case.A case of florid cemento-osseous dysplasia in a 16-year-old Japanese boy is presented. The lesion was unusually large and affected all four quadrants. Progressive increase in the bulk of the lesion was seen.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
2/309. Fibrous dysplasia and ossifying fibroma of the paranasal sinuses.Fibro-osseous lesions involving the paranasal sinuses, the mid-face and anterior skull base are uncommon. In addition, there appears to be no clear pathological or clinical classification that embraces the variety of lesions that exhibit such diverse pathological and clinical behaviour, yet may still be referred to as a fibro-osseous lesion. The diagnosis of fibrous dysplasia and ossifying fibroma is made on a combination of clinical, radiological and pathological criteria. This paper emphasizes the clinical and pathological differences between fibrous dysplasia and ossifying fibroma. The more aggressive clinical behaviour of the latter is highlighted and a more radical surgical approach is recommended. In contradistinction, fibrous dysplasia can exhibit a more benign behaviour and radical surgery is not always justified. A clinicopathological distinction between these two conditions is important from a management perspective despite the fact that they both may be encompassed under the 'umbrella' term fibro-osseous lesion.- - - - - - - - - - ranking = 1.4keywords = dysplasia (Clic here for more details about this article) |
3/309. 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 = 1.2keywords = dysplasia (Clic here for more details about this article) |
4/309. Fibrous dysplasia with intramuscular myxoma (Mazabraud's syndrome). Report of a case and review of the literature.About 30 cases of fibrous dysplasia associated with one or more myxomas (Mazabraud's syndrome) have been reported since 1926. We report a new case in a woman with polyostotic fibrous dysplasia and a myxoma in the left femoral muscle. She also had a history of precocious sexual development and cafe au lait spots, two manifestations whose association with polyostotic fibrous dysplasia defines McCune-Albright syndrome.- - - - - - - - - - ranking = 1.4keywords = dysplasia (Clic here for more details about this article) |
5/309. 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 = 0.2keywords = dysplasia (Clic here for more details about this article) |
6/309. 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 = 1.6keywords = dysplasia (Clic here for more details about this article) |
7/309. 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 = 0.4keywords = dysplasia (Clic here for more details about this article) |
8/309. Familial polyostotic fibrous dysplasia.A case of polyostotic fibrous dysplasia of the craniofacial type is presented, together with substantial evidence that this condition had a genetic basis in this patient. A review of the literature indicates that there is absolutely no previous evidence of a genetic basis to this condition. The possibility that the propositus suffered from polyostotic fibrous dysplasia of the Jaffe or the Albright type was excluded.- - - - - - - - - - ranking = 1.2keywords = dysplasia (Clic here for more details about this article) |
9/309. Florid osseous dysplasia of the mandible: report of a case.In 1986, a 60-year-old African American woman visited the Marquette University School of dentistry with a complaint of a vague, dull pain in her lower left quadrant. tooth No. 19 was extracted 10 years earlier because of extensive decay, and tooth No. 18 had received root canal therapy. A panoramic radiograph revealed the presence of ill-defined, multilocular, mixed (radiopaque-radiolucent) lesions present throughout the lower jaw. She was treated with antibiotics and scheduled for follow-up visits. When the symptoms persisted, tooth No. 18 was re-treated with root canal therapy and a representative biopsy was taken from the left mandibular area. The biopsy showed the presence of chronic osteomyelitis. The patient was treated with antibiotics and was scheduled for periodic check-up visits. In February 1995, she returned with the same symptoms in the left mandible. A panoramic radiograph showed persistence of the mixed radiopaque-radiolucent lesions throughout her mandible; however, the mass on the left side was more radiopaque and had assumed a "cotton wool" appearance. tooth No. 18 was extracted and a biopsy was taken from the area. After correlating the clinical behavior, radiographic appearance, and histopathologic features, a diagnosis of florid osseous dysplasia with osteitis was made. This case represents a classic example of the difficulty in diagnosing fibro-osseous lesions using radiographic interpretation alone and the need to correlate the clinical, radiographic, and histopathologic features to reach a diagnosis. Additionally, the present case clearly shows treatment problems of an otherwise self-limiting condition when secondary involvement with osteomyelitis is also present. A brief description of the conditions that were included in the differential diagnosis and their management is presented.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
10/309. 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 = 1.8keywords = dysplasia (Clic here for more details about this article) |
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