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Orpha number ORPHA55881
Synonym(s) -
Prevalence Unknown
  • Autosomal dominant
  • Sporadic
Age of onset Adolescence / Young adulthood
ICD-10 -
  • C1367554
  • D050398
MedDRA -


Adamantinomas are slow growing malignant bone tumours. These tumours are relatively rare representing between 0.1 and 0.48% of all primary malignant bone tumours, and with just over 500 cases reported in the litterature. The typical presentation of a patient with an adamantinoma is that of a painless swelling on the anterior side of the tibia. This swelling can be present for a long time before medical attention is sought. A second category of patients presents with localised pain at the site of the adamantinoma. However, a significant proportion of patients belong to a third category in which adamantinomas are incidental findings after trauma. Most tumours are located in the middle third of the tibial anterior cortex but involvement of bones other than the tibia and/or the fibula has been reported although is much less common. In rare cases, the tumour can be located purely within the cortex. Cortical thickening, although seen after fracture, is rare due to the slow growth of the tumours. The presence of a soft tissue mass is a rare finding but there have even been scattered reports of adamantinomas solely located within the soft-tissue of the lower limb. The aetiology of these tumours is unknown. On conventional radiography, early stage adamantinomas appear as a cortical lucency without a significant periosteal. However, detection of tumours at this stage is rare and usually fortuitous, with most patients being diagnosed in more advanced stages. The typical tumours are multiloculated cystic/sclerotic lesions with a characteristic `soap bubble like' appearance. As the tumour grows slowly, there is usually some degree of bone expansion. In some cases, the cortex may eventually be disrupted and a soft-tissue component can be found. Although regularly performed, computerised topography does not seem to increase diagnostic accuracy. As seen by conventional radiography, the lesions are mostly eccentric, expansile and cortically located. Like most bone tumours, adamantinomas show low signal intensity on T-1 weighted magnetic resonance imaging (MRI) and high signal intensity on T-2 weighted images. MRI is of value for surgical staging as it provides better delineation of soft tissue and bone marrow involvement. As adamantinomas are rare, diagnosis cannot be made with a high degree of certainty. The main differential diagnosis for adamantinoma is osteofibrous dysplasia (see this term) but fibroma (non-ossifying as well as ossifying), a bone cyst, chondrosarcoma (see this term), Langerhans's cell histiocytosis (see this term) and haemangioendothelioma should also be excluded. Curation can only be achieved after gross total resection with wide resection margins. Chemotherapy and radiotherapy do not play a role in the treatment of adamantinomas. In general adamantinomas have a good outcome. According to the literature, complete remission can be achieved in 70.5% of the cases. However, in 15.8% of cases, the patient eventually died of metastatic disease. Metastases most often occur in the lungs (39.4%) and in the lymph nodes (21.2%), although metastases to liver, bone and brain have been reported.

Expert reviewer(s)

  • Dr Mario MAAS
  • Dr Rick VAN RIJN

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