High grade transformation of salivary gland acinic cell carcinoma with emphasis on histological diagnosis and clinical implications.

Acinic cell carcinoma (ACC) is commonly thought of as a low grade malignant salivary neoplasm, and possibly has the best prognosis of all salivary malignancies with a 10-year survival of almost 90%. High grade transformation (HGT) in these tumours is a relatively rare event but is increasingly being reported. HGT (formerly referred to as dedifferentiation) in acinic cell carcinoma has shown to drastically reduce the survival rates and its recognition is imperative as more aggressive clinical management is needed. We report a case of parotid acinic cell carcinoma in a 82-year old woman where the fine needle aspirate suggested either pleomorphic adenoma or the possibility of carcinoma ex pleomorphic adenoma. Per-operatively it became clear that the facial nerve was involved and the tumour mass was debulked only. The histology showed an acinic cell carcinoma with foci of high grade differentiation (ACC-HGT). We describe the histology of HGT in ACC and the most common differential diagnoses. We emphasise the need of very generous sampling of the tumour, as to recognise any area of high grade transformation, some of which can be very small. A literature review of ACC-HGT as well as HGT in other salivary gland neoplasms is presented. HGT of ACC greatly thus influences the macroscopical and microscopical evaluation of the specimen but also, given the high incidence of metastases and morbidity, carries significant treatment implications.