Papillary cystic acinic cell carcinoma: report of a rare lesion with unusual presentation

Introduction: Acinic cell carcinoma is an uncommon low grade tumour of the salivary glands that constitutes 2.5 to 4% of parotid gland tumours. Acinic cell carcinoma -Papillary cystic variant (ACC-PCV) is histologically composed of tumor with papillary and cystic growth patterns, with varying proportions of one or more cell types. It has been conferred significance because it has a poorer prognosis and is reported to be universally fatal in ten years. Case Report: We present a case of ACC-PCV in a sixteen year old male with unusual unicystic gross appearance, benign cytological picture and characteristic histopathological features .Cystic areas with papillary projection of surrounding cells showing characteristic tombstone or hobnail arrangements were seen. Discussion: The histogenesis and myriad architectural and cellular variations of ACC-PCV have been discussed along with its variegated cytomorphology which may lead to pitfalls in cytodiagnosis. The tumor may pose difficulty in histodiagnosis due to its resemblance to papillary carcinoma of the thyroid.