Osteofibrous dysplasia and adamantinoma: A summary of diagnostic challenges and surgical techniques. (September 2021) - El Beaino, Marc
Abstract: Osteofibrous dysplasia is an indolent benign fibro-osseous tumor, while adamantinoma is a locally aggressive biphasic malignancy with epithelial and fibro-osseous components. Predominantly arising in the tibial diaphysis of children and young adults, both tumors are resistant to chemotherapy and radiation. Wide surgical resection is regarded as the mainstay of therapy for adamantinoma, and limb-salvage reconstructive procedures can achieve good functional outcomes, albeit with non-negligible rates of complications. This review discusses emerging advances in the pathogenesis, histogenesis, and diagnosis of these entities and presents advantages and limitations of the most common surgical techniques used for their management. Highlights: The pathogenesis of osteofibrous dysplasia and adamantinoma is debatable. These fibro-osseous tumors are optimally managed with wide surgical resection. Limb-salvage reconstructions restore function but complications may arise.