Adamantinoma and chordoma are usually addressed consecutively in the textbooks since both are rare, low-grade malignant bone tumors. Neither of these tumors are sensitive to adjuvant treatment modalities and wide resection is the only curative treatment option for both of them. Both have very high local recurrence risk.
Adamantinoma, which is a biphasic tumor with a predilection for tibial diaphysis, must be distinguished from osteofibrous dysplasia, which is a benign condition. Treatment of adamantinoma can be summarized as wide resection followed by intercalary defect reconstruction with preferably biological methods.
Chordoma originates from notochordal remnants in the vertebral column. Orthopaedic oncologists usually treat chordomas of the sacrococcygeal region. Inadequate resections, performed inadvertently or performed with the intent of preserving some function, usually result in early local recurrence. Furthermore, recurring lesions either necessitate more morbid resections or become unresectable. Surgical treatment of chordoma requires multidisciplinary approach. Since distant metastases only appear late in the disease process, patients for whom local control cannot be provided usually experience an agonizing terminal illness period.