Clinicopathological features, diagnosis, and treatment of adamantinoma of the long bones.

Adamantinoma of the long bones is a low-grade, slow-growing, primary malignant bone tumor composed of epithelial cells in a fi brous or osteofi brous stroma.1,2 It has a wide range of histological patterns.3-18 Despite the considerable range of each histologic pattern, most current studies have shown that long bone adamantinoma appears to be of an epithelial nature. The tibial lesion’s name comes from its somewhat similar histologic appearance to the more common odontogenic adamantinoma of the jaw bones.18,19 Despite the histological similarities there is no proof that these tumors have a similar histiogenetic origin.19 Long bone adamantinomas can be divided in two main groups with distinct histological and radiographic features. The fi rst–classic adamantinoma–is characterized by an abundance of tumor cells and a destructive growth pattern. The second, differentiated adamantinoma, is characterized histologically by a predominance of an osteofi brous dysplasia-like pattern with a small, inconspicuous component of epithelial tumor elements.12,18,19 Radiographically, classic adamantinomas are either intracortical or associated with complete cortical disruption, intramedullary involvement, and expansion beyond the periosteum into adjacent soft tissues, while differentiated adamantinomas are intracortical and often multicentric lesions.12,19,20 This article discusses the epidemiology, clinicopathological and imaging features, diagnosis, treatment, and prognosis of adamantinoma of the long bones. EPIDEMIOLOGICAL DATA Adamantinoma of the long bones is a rare tumor that accounts for 0.3%-1% of all Clinicopathological Features, Diagnosis, and Treatment of Adamantinoma of the Long Bones