Yonsei Med J.  2007 Jun;48(3):502-510. 10.3349/ymj.2007.48.3.502.

Treatment of Osteofibrous Dysplasia and Associated Lesions

• Hahn SB
• Kim SH
• Cho NH
• Choi CJ
• Kim BS
• Kang HJ

• KMID: 724107
• DOI: http://doi.org/10.3349/ymj.2007.48.3.502

Abstract

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.

Keyword

Osteofibrous dysplasia; osteofibrous dysplasia like adamantinoma; adamantinoma

MeSH Terms

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

Figure

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  Fig. 1 (A) Initial radiographs, which were diagnosed as osteofibrous dysplasia, showed an extensive osteolytic lesion with a severe anterior bowing deformity of left tibia. (B) Radiographs after segmental resection and free vascularized fibular graft. (C) Postoperative 14 years, this patient had an osteolytic round lesion on the junction of the grafted fibula and tibia, thus suggesting a recurrence. (D) The recurrent lesion was excised and reattached after pasterization. (E) Photomicrographs representing the lesion area and showing the large nests of epithelial neoplastic cells in the dense sclerotic stromal tissue, which are compatible with classic AD (H & E × 200). (*; epithelial cells, #; stromal tissue) (F) Cells showing a positive immunohistochemical staining for cytokeratin (× 100). (G) The last follow-up radiograph showed no evidence of recurred lesion (4 years and 11 months after the second operation).

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  Fig. 2 (A) Initial radiographs showed the extensive osteolytic lesion which was rapidly growing. (B) Radiographs after a wide resection and free vascularized fibular grafts. (C) In this patient, the osteofibrous dysplasia had recurred 12 years after their first operation. Thus, they were treated with a segmental resection and pasteurization. (D) The biopsy of the tibial lesion illustrates a fibrous lesion of bone with the bone formation surrounded by abundant osteoblasts, which is consistent with osteofibrous dysplasia (H & E × 200). (*; stromal tissue, black arrow; osteoblast, white arrow; osseous trabecule). (E) The last follow-up radiograph taken 5 years and 1 month after the secondary operation. Recurrence is not evident in this micrograph.

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