Adamantinoma is a biphasic tumor, with a low potential for malignancy, characterized by clusters of epithelial cells surrounded by a relatively bland spindle-cell osteofibrous component. The aim of the present study was to review the updated data regarding epidemiology; pathogenesis; clinical presentation; radiological, histopathological and ultrastructural findings; and treatment options of adamantinoma. In X-ray, it is usually seen as an eccentric and sometimes central, lobular, lytic lesion with sclerotic margins of overlapping radiolucency, and a characteristic 'soap-bubble' appearance. Magnetic resonance imaging seems to be the most appropriate examination for differential diagnosis between adamantinoma and other skeletal tumors. Histologically, adamantinoma is identified as classic adamantinoma or osteofibrous-like adamantinoma. Classic adamantinoma is classified into four patterns of growth: Basaloid, tubular, spindle cell, and squamous. The preferable treatment of this tumor type is en bloc resection within wide operative margins, which may include suspicious regional lymph nodes, with limb reconstruction and limb salvage.According to the definition of the World Health Organization, adamantinoma is a miscellaneous malignant biphasic tumor and its characteristics are clusters of epithelial cells surrounded by a relatively bland spindle-cell osteofibrous component (1, 2). It is a tumor with low frequency (as it occurs in less than 0.5% of all primary skeletal tumors), in terms of age most often developing between the age of 20 and 50 years, and it is more common in men. It may be located in several bones; however, the mid shaft of the tibia, with or without fibula involvement, is its most common location (3-5). The main symptom reported by patients is pain (6). Treatment of adamantinoma includes extensive resection and reconstruction of bone defect with the use of autograft, allograft, bone transport, and endoprosthesis (7).In our previous article, we presented the epidemiological, clinical, radiological and pathological features as well as treatment options of adamantinoma (4). The aim of the present study was to review the updated data regarding history, incidence, epidemiology, origin and pathogenesis, clinical features, as well as radiological, histopathological and ultrastructural findings, new treatment strategies and prognosis of adamantinoma.
The History of AdamantinomaAlthough adamantinoma is an unusual bone tumor, its origin has been debated for almost a century. The term adamantinoma comes from the ancient Greek word adamantinos, which means very hard, as the root word adamas means diamond. The first reported case of a primary skeletal tumor with epithelial characteristics, observed in the ulna, was by Maier (8) in 1900, while in 1913, a physician from Germany, called this tumor 'adamantinoma' as he observed that during embryonic development adamantine 3045 This article is freely accessible online.