Adamantinoma of long bones. A clinicopathologic study of 85 cases

A study of 85 adamantinomas of long bones revealed that 70 were in the tibia (11 of which also involved the fibula), six were in the femur, three were in the ulna, two were in the humerus, two were in the fibula, one was in the radius, and one arose in the soft tissue anterior to the tibia. Most patients presented with pain and swelling and were aged 10 to 30 years. The histologic appearance was that of epithelial islands in a fibrous stroma, usually with a prominent vascular pattern and a transition between the two. Twenty-six (31%) patients had recurrent local disease, 13 (15%) developed lung metastasis, and six (7%) had lymph node metastasis. Nine patients with lung metastasis had preceding recurrent local disease. Risk factors for recurrent or metastatic disease included male sex, pain, symptoms of less than 5 years' duration, and initial treatment by biopsy, curettage, excision, or resection. The only histologic feature associated with an increased recurrence rate was lack of squamous differentiation. Eleven patients died of their disease, and seven are alive with metastatic disease. Forty-one patients were still alive without disease 1 month to 47 years after treatment. Our results indicate that amputation or, when technically feasible, wide en bloc resection is the treatment of choice. All patients require long-term follow-up for evidence of local recurrence or lung metastasis.