Adamantinoma/surgery* ; Bone Neoplasms/surgery* ; Humans ; Neoplasms ; Tibia

Osteofibrous dysplasia (OFD)-like adamantinoma is a rare skeletal tumor that is characterized by the predominant OFD-like pattern with scattered epithelial nests. Adamantinoma shares clinical features (the majority of lesions in the tibia and the prevalent age group), radiologic findings (radiolucency with sclerotic shadow), and pathologic similarities (particularly the presence of scattered cytokeratin-positive stromal cells) with OFD. We describe a case of OFD-like adamantinoma. Epithelial cell nests express the epithelial membrane antigen, pancytokeratin, CK14, and collagen type IV. Ultrastructurally, the oval to spindle cells in the epithelial foci had abundant tonofilaments, and well-formed desmosomes with dense plaques, of which well preserved desmosomes are demonstrated for the first time in OFD-like adamantinoma. These immunohistochemical and ultrastructural findings further support that the origin of epithelial cells of classic and OFD-like adamantinoma are epithelial cells transformed from fibroblastic cells in the proliferating osteofibrous tissue.
Adamantinoma* ; Collagen Type IV ; Desmosomes ; Epithelial Cells ; Fibroblasts ; Fibroma, Ossifying ; Immunohistochemistry ; Intermediate Filaments ; Mucin-1 ; Tibia

Humans ; Adamantinoma/surgery* ; Bone Diseases, Developmental ; Bone Neoplasms/surgery* ; Tibia/surgery*

Ossifying fibroma is not a well recognized entity, and only rarely affects the long bones. The main differential diagnosis is with fibrous dysplasia and with adamantinoma, non-ossifying fibroma of the long bone. Ossifying fibroma seldom has even a moderate tendency to progress during childhood, but it recurs frequently after curettage or subperiosteal resection. Any progression of the lesion comes to an end after puberty. So, surgery should be delayed as long as possible. But, if the lesion is rapidly progressive, or if a patient has repeated fractures, it would be necessary to resort to wide extraperiosteal resection. We have experienced 7 cases of ossifying fibroma from Octover 1979 to November 1988. The results were as follows; 1. The male and female ratio, was 5 to 2 and six patients were at their first decade, and one patient was 13 years old. 2. The lesion site was six cases in the tibia, one case in the fibula. 3. In six patients, we had done wide resection with free-vasculaized fibular graft in five cases and wide resection of the distal one-third of the fibula in one case. Two patients among them had recurrence. One case of recurrence was performed incomplete wide resection with free vascularized fibular graft because the lesion was too close to the distal epiphysis of the tibia.
Adamantinoma ; Adolescent ; Curettage ; Diagnosis, Differential ; Epiphyses ; Female ; Fibroma ; Fibroma, Ossifying ; Fibula ; Health Resorts ; Humans ; Male ; Puberty ; Recurrence ; Tibia ; Transplants

Adamantinoma ; pathology ; Carcinoma, Mucoepidermoid ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Diagnosis, Differential ; Humans ; Male ; Mandibular Neoplasms ; diagnostic imaging ; pathology ; surgery ; Middle Aged ; Tomography, X-Ray Computed

PURPOSE: We evaluated the results of allograft reconstruction following wide resection of malignant bone tumors in long bone, retrospectively. MATERIALS AND METHODS: Seven patients were included. The mean age was 44 years old. Male was 4 cases, and female was 3 cases. Mean follow-up period was 38 months. The mean Musculoskeletal Tumor Society (MSTS) score at final follow-up was evaluated. Postoperative complications were evaluated via periodic radiologic follow-up. Oncologic results were analyzed at final follow-up. RESULTS: The primary malignancies occurred at femur in 5 cases, humerus in 1 case and tibia in 1 case. Pathologic diagnoses were osteosarcoma in 4 cases, multiple myeloma in 2 cases and adamantinoma in 1 case. Mean length of allograft was 165 mm. Fixations of allograft were intramedullary nailing with additional plate in 4 cases, intramedullary nailing in 2 cases, and screw fixation in 1 case. Mean time to union was 14.5 weeks. Mean MSTS score at final follow-up was 20 (67%). Postoperative complications were nonunion in 3 cases, implant failure in 1 case, and infection in 1 case. Oncologic outcomes were continuous disease free in 5 cases and alive with disease in 2 cases at final follow-up. Autologous bone graft and hemi-cortical onlay graft were performed in 2 cases of nonunion. CONCLUSION: Allograft reconstruction following wide resection of malignant bone tumors in long bone was effective surgical option. However, the possibility of nonunion between host bone and allograft should be considered.
Adamantinoma ; Allografts* ; Diagnosis ; Female ; Femur ; Follow-Up Studies ; Fracture Fixation, Intramedullary ; Humans ; Humerus ; Inlays ; Male ; Multiple Myeloma ; Osteosarcoma ; Postoperative Complications ; Retrospective Studies ; Tibia ; Transplants

Adamantinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adult ; Diagnosis, Differential ; Female ; Femur ; Follow-Up Studies ; Humans ; Humerus ; Ilium ; Keratins ; metabolism ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Retrospective Studies ; Sarcoma, Ewing ; pathology ; Sarcoma, Synovial ; pathology ; Tibia ; Tomography, X-Ray Computed ; Young Adult

PURPOSE: To report long term treatment outcomes of osteofibrous dysplasia and association with adamantinoma. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Adamantinoma/metabolism/pathology/*surgery ; Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Fibrous Dysplasia of Bone/metabolism/pathology/*surgery ; Fibula/chemistry/radiography/surgery ; Humans ; Immunohistochemistry ; Keratins/analysis ; Male ; Middle Aged ; Tibia/chemistry/radiography/surgery