The case of an infant born with a large polypoid tumour arising from t
he mouth is described. The tumour had the histological, immunohistoche
mical and ultrastructural phenotype of an extrarenal malignant rhabdoi
d tumour and followed an aggressive clinical course. This is one of th
e few reported cases of malignant rhabdoid tumour to present at birth.
The oral tumour was associated with a mass in the posterior cranial f
ossa. This was most likely to be a simultaneous second primary tumour.
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