bromochloroacetic-acid and Fibrous-Dysplasia-of-Bone

In view of the still disputed relationship between adult adamantinoma and osteofibrous dysplasia in children, a unique case of adamantinoma, indicating a direct relationship between the two lesions, is presented with a review of the literature. The patient was a six-year-old boy who complained of pain and swelling in the left lower leg. Roentgenographs showed a loculate osteolysis surrounded by sclerosis within the cortex of the tibial shaft that would be typical of osteofibrous dysplasia. Although an osteofibrous-dysplastic component predominated histologically, some small islands of epithelial cells were scattered throughout the lesion. Immunohistochemically, the tumor cells of these epithelial islands gave a constant positive reaction for cytokeratin as well as vimentin, while the stromal cells in the osteofibrous dysplasia-like lesion were positive for vimentin only. This type of lesion is recorded in the Bone Tumor Registry of Westphalia at a rate of 8.3% for osteofibrous dysplasia, and of 25% for adamantinoma. A review of the literature, yielding reports with remarkable uniformity on 14 cases beyond the present one, suggests the existence of a separate clinicopathologic entity to be called juvenile intracortical adamantinoma with predominant osteofibrous dysplasia-like features, and which might be a regressing form of adamantinoma specific in childhood.

Topics: Ameloblastoma; Bone Neoplasms; Child; Fibrous Dysplasia of Bone; Humans; Immunohistochemistry; Keratins; Male; Tibia; Vimentin

To report long term treatment outcomes of osteofibrous dysplasia and association with adamantinoma.. From January 1984 to July 2001, 14 patients with osteofibrous dysplasia were followed for an average of 108 months (78 to 260 months). Our patient group consisted of 6 men and 8 women, with a mean age of 13.9 years (2 to 65 years). We reviewed the clinical and pathological features of all 14 patients.. Thirteen patients had a lesion in the tibia, while one patient had lesions in both the tibia and the fibula. Initial treatments were observation after biopsy (6 patients), curettage with or without a bone graft (3 patients), resection followed by a free vascularized fibular bone graft (4 patients), or resection and regeneration with the Ilizarov external fixation (1 patient). Curettage was performed on 6 patients due to recurrence or progression after the initial treatment. Among these patients, one was diagnosed with AD from the biopsy of the recurrent lesion. This patient was further treated by segmental resection and pasteurization. After the initial pathology slides of the 13 patients were reviewed with immunohistochemical cytokeratin staining, one patient diagnosis was changed from osteofibrous dysplasia to osteofibrous dysplasia-like adamantinoma.. Some patients with osteofibrous dysplasia require close observation because of the high association risk between osteofibrous dysplasia and adamantinoma, Immunohistochemical staining may be helpful in differentiating these two diagnoses.

Topics: Adamantinoma; Adolescent; Adult; Aged; Child; Child, Preschool; Female; Fibrous Dysplasia of Bone; Fibula; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Radiography; Tibia

This report describes five cases of osteofibrous dysplasia-like adamantinoma of the tibia in young patients ranging from ages 4 1/2 to 14 years. Radiologically and histologically, these cases were indistinguishable from osteofibrous dysplasia of bone, and no epithelial cells were recognized on routine staining. However, epithelial differentiation was seen in the form of scattered keratin-positive cells in all five cases, and tonofilaments in four cases. The patients were treated by curettage, and three had recurrences. Follow-up showed no progression to classic adamantinoma. Osteofibrous dysplasia-like adamantinoma is a special histological type of adamantinoma that affects children and adolescents. It differs from classic adamantinoma in that it lacks conspicuous nests and masses of epithelial cells, and the prognosis after conservative treatment is generally good. Recent publications suggest that osteofibrous dysplasia-like adamantinoma is a precursor of classic adamantinoma. In a comparative study of three cases of classic adamantinoma, we found, in the fibroblastic stroma of the tumors, spindle epithelial cells that were indistinguishable from the epithelial cells of osteofibrous dysplasia-like adamantinoma. This finding suggests that there is an overlap between these conditions. Four additional cases of osteofibrous dysplasia of the tibia from our files lacked epithelial differentiation. It is most likely that osteofibrous dysplasia is part of the morphologic spectrum of adamantinoma.

Topics: Adolescent; Adult; Bone Neoplasms; Child; Child, Preschool; Female; Fibrous Dysplasia of Bone; Humans; Immunoenzyme Techniques; Intermediate Filaments; Keratins; Male; Neoplasms, Glandular and Epithelial; Tibia

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.

Topics: Ameloblastoma; Antibodies, Monoclonal; Bone Neoplasms; Female; Fibrous Dysplasia of Bone; Fibula; Humans; Immunohistochemistry; Keratins; Male; Tibia; Vimentin

A clinicopathological and immunohistochemical study of 12 cases of osteofibrous dysplasia (OFD), two cases of differentiated adamantinoma, and five cases of adamantinoma of long bones is presented. Although OFD and differentiated adamantinoma showed similar radiologic findings, differentiated adamantinoma was more likely to be a recurrent lesion than osteofibrous dysplasia and seemed to require a more extensive surgical procedure. Immunohistochemically, cytokeratin- and vimentin-positive cells were seen in both OFD and differentiated adamantinoma. The positive cells were scattered in the former, and were both scattered and nest-like in the latter. Both these lesions, however, were negative for epithelial membrane antigen. Excluding two cases of Ewing-like adamantinoma, the other three cases of adamantinoma were also positive for cytokeratin and vimentin. These results suggest that these three lesions share the same histogenetic origin. The two cases of Ewing-like adamantinoma differ from tibial adamantinoma in their radiological, histological and immunohistochemical aspects, and seem to constitute a distinct variant of adamantinoma with a different histogenesis.

Topics: Adolescent; Adult; Ameloblastoma; Bone Neoplasms; Cell Nucleus; Child; Cytoplasm; Epithelium; Female; Fibrous Dysplasia of Bone; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Osteoblasts; Osteoclasts; Radiography; Tibia; Vimentin