Palatal Polymorphous Adenocarcinoma with High-Grade Transformation: A Case Report and Literature Review

Polymorphous adenocarcinoma (PAC) is the second most common intraoral malignant neoplasm of the minor salivary glands. However, it is very rare for PAC to show high-grade transformation (HGT) and to our knowledge, the English literature only seven reported cases. HGT tends to be observed when PAC recurs, and it is extremely rare to be seen at initial presentation. Here we report a 43-year-old Japanese male patient with PAC of the right palate showing HGT at initial presentation. Histopathologically, the tumor was characterized by a prominent solid and papillary-cystic growth pattern, with nuclear atypia and necrosis in area of HGT. The immunohistochemical staining pattern was consistent with PAC, as the tumor cells showed diffuse positivity for cytokeratin, vimentin and S-100, and focal positivity for bcl-2, alpha-SMA and EMA. The tumor cells in HGT areas were markedly positive for AR and Ki-67 (about 40%/HPF), and also focally positive for cyclin D1 and p53, whereas HER2/neu, ER, PgR, p63, D2-40, GCDFP-15, and mitochondria were negative. Here we present a very rare case of palatal PAC with HGT at initial presentation.