Pathological Insights into Osteofibrous Dysplasia and Adamantinoma
Summary
Osteofibrous dysplasia and adamantinoma represent ends of a spectrum of fibro-osseous lesions affecting long bones, most frequently the tibia. Osteofibrous dysplasia is a benign, self-limiting fibro-osseous proliferation, whereas adamantinoma is a rare low-grade malignant neoplasm characterised by epithelial islands within a fibrous stroma. Advances in histopathology, immunohistochemistry and imaging have refined the understanding of their interrelationship, notably the concept of osteofibrous dysplasia-like adamantinoma as an intermediate lesion. Radiologically, osteofibrous dysplasia features well-circumscribed intracortical lucencies with sclerotic margins, whereas adamantinoma exhibits more aggressive cortical destruction and periosteal reaction. Microscopically, epithelial cell nests highlighted by cytokeratin staining distinguish adamantinoma from its benign counterpart. Molecular studies have begun to identify pathways implicated in epithelial–mesenchymal interactions, but the precise aetiology remains elusive. Clinically, differentiation is critical as management ranges from conservative observation for osteofibrous dysplasia to wide resection for classic adamantinoma. Emerging consensus supports long-term surveillance given the potential for local recurrence and, in rare cases, pulmonary metastasis.
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Pathological Insights into Osteofibrous Dysplasia and Adamantinoma publication trend
The graph below shows the total number of articles in pathological insights into osteofibrous dysplasia and adamantinoma across all publications each year (not limited to Nature Index journals).
Technical terms
Osteofibrous dysplasia: Benign fibro-osseous lesion of the cortical bone, typically affecting the tibia in children.
Adamantinoma: Rare low-grade malignant bone tumour characterised by epithelial cell nests within fibrous stroma.
OFD-like adamantinoma: Intermediate lesion with histological features overlapping osteofibrous dysplasia and classic adamantinoma.
Curettage: Surgical removal of lesion tissue by scraping from the bone cavity.
Wide resection: Surgical excision of a tumour with a margin of healthy tissue to minimise recurrence.
References
- Adamantinoma: A clinicopathological review and update. Diagnostic Pathology (2008).
- Outcome of osteofibrous dysplasia-like versus classic adamantinoma of long bones: a single-institution experience. Journal of Orthopaedic Surgery and Research (2020).
- Osteofibrous dysplasia-like adamantinoma: A case report and literature review. Frontiers in Oncology (2022).
- Clinical outcomes in patients with adamantinoma: Report from the bone and soft tissue tumor registry in Japan. Journal of Surgical Oncology (2024).
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