Adamantinoma

Abstract

Adamantinoma is one of the rarest low-grade malignant bone tumours, representing less than 1% of them. Fisher in 1913 named this tumour adamantinoma because of its similarity to ameloblastoma of the jaw. It usually arises in the center of long bones, and 97% of all reported cases were in long tubular bones and mainly in the tibial mid shaft (80-85%). Other long bones not uncommonly affected are the humerus, ulna, femur, fibula and radius. Ribs, spine, metatarsal and carpal bones are very rarely affected. The symptoms are not specific but most frequently the patient complains about swelling, redness, pain and sensitivity of the bone where the tumour is located. Young males are more prone to develop adamantinoma than females. The tumour usually spreads to the lungs, the regional lymph nodes, or other bones. Wide tumour excision and limb salvage reconstruction surgery, or an amputation, are the current surgical treatment options. Radiotherapy and chemotherapy have not been shown to be effective modalities of treatment.