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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.

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Surgical resection is the only option of cure for patients with metastatic colorectal cancer. Risk of recurrence after metastasectomy is around 75%. Use of adjuvant chemotherapy after metastasectomy is controversial.AimTo address whether adjuvant systemic therapy after colorectal cancer metastasectomy offers any survival benefit compared with surgery alone.MethodsSystematic review of literature and meta-analysis of all available randomised evidence. Relative hazards (RHs) were summarised across trials and heterogeneity was assessed with the Q and I2 statistics.ResultsFive trials were eligible. Three trials, all using single-agent fluoropyrimidine chemotherapy, presented data valuable for analyses. 482 patients were included in the meta-analysis: 238 randomly assigned to receive postoperative chemotherapy and 244 to metastasectomy only. We found no overall survival (OS) benefit with the use of postoperative single-agent fluoropyrimidines compared with surgery alone, even if a trend for benefit was observed (relative hazard (RH)=0.781, 95% CI 0.593 to 1.030, p=0.080). Significant disease-free survival benefit with the use of postoperative chemotherapy was observed (RH=0.645, 95% CI 0.509 to 0.818, p=0.001). No quality of life (QL) data were available. All trials showed accrual delay, two stopped and one recruiting after 10 years. Long follow-up needs were evidenced since OS curves split only after 3.5 years.ConclusionsNo OS benefit was documented from the use of postoperative monochemotherapy. Metastasectomy alone continues to be the standard of care. Combination chemotherapy regimens should be evaluated along with QL assessment in future trials appropriately designed for long-term accrual and follow-up.