Polymorphous low-grade adenocarcinoma--a rare and aggressive entity in adolescence

Polymorphous low-grade adenocarcinoma (PLGA) is an uncommon tumour that usually affects the minor salivary glands, particularly in the palate. It is rare in young patients, and here we report a case in a teenage girl. She presented at the age of 16, although the lesion had been noticed 2 years previously. The tumour showed histopathological features of PLGA, but recurred locally, behaved aggressively, and ultimately metastasised to cervical lymph nodes. This was accompanied by an altered histological picture, with a papillary cystic pattern and necrosis becoming progressively more prominent. PLGA is not always a low-grade lesion and some tumours, notably those with a papillary cystic growth pattern, may require more aggressive treatment.