1/361. 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. ( info) |
| 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. ( info) |
3/361. 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. ( info) |
4/361. 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. ( info) |
5/361. 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. ( info) |
6/361. 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. ( info) |
7/361. 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. ( info) |
8/361. Fibro-osseous lesion of the mandible mimicking chronic osteomyelitis. An account is given of the clinical, radiologic, and pathologic, features of a special form of fibro-osseous lesion of the mandible. The material consists of fifteen cases (eleven females and four males). Two cases are described in detail. Together with Case 1, a probably unique series of radiographic pictures is presented, showing the development of the disease. ( info) |
9/361. 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. ( info) |
10/361. Brown tumor of the sternum: a potential source of false-positive Tl-201 and Tc-99m subtraction imaging in the mediastinum. Tl-201 and Tc-99m subtraction imaging is a well-established technique for detecting parathyroid adenomas. We report a case of a brown tumor of the upper sternum mimicking an ectopic mediastinal parathyroid adenoma on Tl-201 and Tc-99m subtraction imaging in a patient with hyperparathyroidism. This brown tumor showed Tl-201 uptake but not Tc-99m uptake and thus did not subtract out in this scan. ( info) |