Adamantinoma in childhood: report of six cases and review of the literature.

BACKGROUND: Adamantinoma is a rare slow-growing malignant bone tumour.
OBJECTIVE: To describe the imaging appearances of six childhood cases and review the published literature.
MATERIALS AND METHODS: The database of the Working Group on Paediatric Oncology, Academic Medical Centre/Emma Children's Hospital Amsterdam, was searched for cases of adamantinoma. Additionally a literature study was performed to identify cases of adamantinoma in childhood.
RESULTS: We identified six local cases of adamantinoma of the long bones, two boys (age 3 and 8 years) and four girls (mean age 8.8 years, range 3.0-14.0 years). The location of the tumour was the tibia in five and the tibia and fibula in one patient. In two patients initially a different diagnosis was made, which led to a delay in appropriate treatment. None of the children showed pulmonary metastases and all underwent total gross resection. On follow-up (mean 6.1 years, range 1.6-12.0 years) all children remained disease-free. Besides a discussion of our six patients, imaging features, histopathology, surgical approach and a literature review of childhood adamantinomas is presented.
CONCLUSION: Although the incidence of adamantinoma is low, it is important to recognize this rare bone tumour, since in the early stages of the disease adequate treatment will result in an excellent prognosis.