Publication:

Intraosseous polymorphous adenocarcinoma of mandible

Polymorphous Adenocarcinoma (PAC) is a type of salivary gland tumor commonly arising in the minor salivary glands with palate being the most frequent site of origin. PACs harbor recurrent PRKD1 E710D hotspot mutations in >70% of cases. It has propensity of malignant transformation along with locoregional or distant metastasis. However, the latter is very rare entity.  Often challenging for both the pathologist and surgeon is the histologic difference between other conditions like pleomorphic adenoma, adenoid cystic carcinomas and adenocarcinoma not otherwise specified. Interestingly, very rare sites of occurrence may herald the rarity, therefore the intraosseous origin in the maxilla and the mandible are seldom encountered. One such very rare case of PAC of the mandible with extensive destruction of hemimandible and the areas in the vicinity of the tumour with nodal and distant bony metastasis described here.