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.


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.


CONCLUSIONS
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.
Van Rijn
Rick
R
Department of Radiology, Academic Medical Center Amsterdam, Meibergdreef 9, Amsterdam, Zuid-Oost 1105 AZ, The Netherlands. r.r.vanrijn@amc.uva.nl
Bras
Johannes
J
Schaap
Gerard
G
van den Berg
Henk
H
Maas
Mario
M
D050398:Adamantinoma / Q000175:diagnosis* / Q000601:surgery; D000293:Adolescent; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007866:Leg; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed