Dysgenetic polycystic disease of the parotid gland: Report of case

Dysgenetic polycystic disease (DPD) is a rare non-neoplastic cystic condition that affects the parotid glands. It is considered to be a developmental malformation of the duct system, Few examples have been recorded in the Literature. In 1981, Seifert et al(1) first described the clinical and histologic aspects. An earlier article by Mihalyka(2) reported a possible case without providing definitive histologic evidence. This congenital disease is characterized by replacement of a lobule of the parotid gland by multiple epithelial-lined cystic spaces arising from the intercalated ducts. The developmental nature is based on the lesion being confined to a lobule and the lack of any signifi cant clinical or histologic evidence of inflammation or alterations of-the remaining ducts. (1,3-5) Seifert et al(1) theorized that the cysts are caused by disturbed embryonal development of the intercalated duct system, paralleling dysgenic polycystic disease of other parenchymal organs such as the pancreas and kidney, It also appears that this condition may be inherited, with an autosomal dominant pattern in females. Clinically, DPD may develop during childhood or may not appear until adulthood. Typically the lesion presents as an asymptomatic, intermittent swelling of the involved gland, which in most cases is bilateral, No alteration of other lobules of the salivary gland or of excessive or reduced salivation is usually evident. Because of the rarity of this condition, and the slight variation in histologic features, an additional case of DPD is presented to help further the understanding and recognition of this rare disorder.