OBJECTIVE: To study the clinical manifestation, radiograph features, pathology, diagnosis and treatment of fibrous dysplasia in cranial bone. METHOD: A case of fibrous dysplasia involving sphenoid and occipital is reported and literature were reviewed. RESULT: The most common complaint were headache, proptosis, diplopia, or visual changes. Distinguishing features of fibrous dysplasia on CT include "ground-glass" appearance, bone fiber anisotrophy, aneurysmal bone cyst formation and thickness of the cranial cortices. Fibrous dysplasia could be exactly diagnosed by pathology. Computed tomography was also a choice for diagnosis. CONCLUSION Fibrous dysplasia involving the cranial bone can present in myriad ways. Modern imaging modalities and histopathologic analysis is required to make accurate diagnosis. Surgery, particularly in a challenging region such as sphenoid and occipital bone, should preserve the existing function for the patients with functional impairment or a cosmetic deformity.
Adult ; Fibrous Dysplasia, Polyostotic ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Male ; Occipital Bone ; Radiography ; Sphenoid Bone

We report a case of adamantinoma of the tibia resembling fibrous dysplasia. The patient was a 55-year-old male, and complained of pain in the right lower leg. Roentgenographs showed a well demarcated osteolytic lesion with small foci of calcification and septation within the diaphysis of the distal tibia. The cortex was partially disrupted. Histologically, initial biopsy specimen showed fibrous connective tissue and trabeculae of immature woven bone, strongly suggestive of fibrous dysplasia. The lesion recurred and the second biopsy revealed nests of spindle cells and tubular epithelial structures embedded in granulation type-fibrous tissue. Immunohistochemically, both the nests of spindle cells and the tubular structures gave a positive reaction for cytokeratin. The present case emphasizes once again that histological diagnosis of fibrous dysplasia of the tibia should be made carefully with exclusion of the possibility of adamantinoma.
Ameloblastoma/*pathology/radiography ; Bone Neoplasms/*pathology/radiography ; Case Report ; Fibrous Dysplasia of Bone/*pathology/radiography ; Human ; Male ; Middle Age ; Tibia/*pathology/radiography

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

OBJECTIVEto study the clinicopathologic features and differential diagnosis of low-grade central osteosarcoma (LGCOS).

METHODSnine cases of LGCOS were retrieved from the archival consultation files. The clinical, radiologic and pathologic features were analyzed, with literature review.

RESULTSthe mean age of the patients was 31 years. The male-to-female ratio was 3:6. All of the patients presented with painful mass and/or swelling. The sites of involvement included thigh (n = 4), tibia (n = 1), fibula (n = 1), cervical vertebra (n = 1), lumbar vertebra (n = 1) and maxilla (n = 1). Radiologic examination showed mixed lytic/blastic lesions with soft tissue shadow in 5 cases and associated periosteal reaction in 3 cases. The tumors were treated by surgical excision, with no adjuvant therapy given. The duration of follow up ranged from 2 to 43 months. Four cases had recurrence which occurred at 8 to 25 months after the operation. Gross examination showed that the tumors were fragmented on submission in 5 cases and en bloc in 4 cases. They had solid and firm cut surface, with various degree of grittiness. Histologically, LGCOS was characterized by the presence of hypocellular fibroblastic stroma associated with focal osteoid production. The spindly tumor cells showed mild degree of nuclear pleomorphism, with occasional mitotic figures demonstrated in all of the 9 cases. The newly formed neoplastic woven bone did not have any osteoblastic rimming. The bony trabeculae were slender and seam-like. Parallel arrays of woven bone were seen in 6 cases. Some of the bony trabeculae appeared irregularly branched and curved. The tumor cells permeated adjoining pre-existing bony trabeculae and bone marrow in all cases. Three cases also showed soft tissue involvement.

CONCLUSIONSLGCOS often posses important diagnostic pitfalls due to the relatively bland-looking tumor cell morphology and associated large woven or longitudinal seams of lamellar-like bone. Thorough understanding of the histologic features, when coupled with clinical and radiologic findings, are essential in arriving at a correct diagnosis.

Adolescent ; Adult ; Bone Neoplasms ; diagnostic imaging ; pathology ; surgery ; Diagnosis, Differential ; Female ; Fibrous Dysplasia of Bone ; pathology ; Fibula ; diagnostic imaging ; Histiocytoma, Benign Fibrous ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Osteosarcoma ; diagnostic imaging ; pathology ; surgery ; Radiography ; Radionuclide Imaging ; Reoperation ; Thigh ; diagnostic imaging ; pathology ; Young Adult