Dysgenetic polycystic disease of the minor salivary glands of the tongue

Abstract

Here we present a case of a 55-year-old male who presented with a 0.5 × 0.5 mm tissue-colored nodule of the lateral tongue. After histopathologic analysis, a diagnosis of dysgenetic polycystic disease was made. Dysgenetic polycystic disease, which has also been called “polycystic disease” or “polycystic parotid disease,” is a very rare developmental abnormality affecting the salivary gland duct system. The histology is distinct, showing numerous closely-packed cystic spaces lined by a bilayer of salivary-type epithelial cells and myoepithelial cells with occasional spherolith-like calcifications. This entity has been reported in only 20 patients previously. In previously reported cases, this lesion presents bilaterally affecting the parotid gland or rarely the submandibular gland. The present case report details the first time dysgenetic polycystic disease is found affecting the minor salivary glands of the tongue. Immunohistochemical findings are presented and include positivity for AE1/AE3 in luminal cells, positive p63 in the abluminal cells, scattered positive SMA in the abluminal cells, PAS-positive diastase resistant spheroliths, and negativity for Congo red, progesterone receptor, mammaglobin, S100.