Is there a link between osteofibrous dysplasia and adamantinoma?

Because of the relative frequency of osteofibrous dysplasia (OFD) and the gravity of adamantinoma, it is important to know whether there is a link between these two entities. A young boy had been followed from the age of 5 years for OFD of the right tibia. At the age of 10, biopsy performed because of pain, revealed OFD-like adamantinoma. Surgery was undertaken, with en bloc proximal tibial resection of 14 cm and reconstruction by free vascularized fibula and internal fixation. This observation illustrates the risk of evolution of OFD-like adamantinoma, showing the same unfavorable evolution as classic adamantinoma. Strict surveillance is mandatory in OFD, with systematic biopsy in case of onset of pain or increased tumor volume.