Papillary cystic variant of the acinic cell adenocarcinoma

The papillary cystic variant of the acinic cell adenocarcinoma is a rare salivary gland tumour, which may behave in an unpredictable manner and exhibit non-specific histological features. Its diagnosis is important as it may prognosticate for a poorer outcome and it has been reported to universally fatal. We present a case of this unusual tumour and discuss the diagnostic and management difficulties.