Polycystic dysgenetic disease of the parotid gland (PDDP) is a rare benign condition of the parotid gland. The disorder presents often in childhood or young adulthood, but may occur later in life. It occurs most commonly in females. ... Polycystic dysgenetic disease of the parotid gland (PDDP) is a rare benign condition of the parotid gland. The disorder presents often in childhood or young adulthood, but may occur later in life. It occurs most commonly in females. Features include fluctuating and nontender swelling of the parotid gland bilaterally, without defects in salivary function. Histology shows replacement of the lobular portion of the parotid gland by multiple epithelial-lined cysts arising from the intercalated ducts. The cysts often contain altered salivary secretions, including spheroliths or microliths; eosinophilic congophilic deposits have also been described. Chronic inflammation is not present. The condition is thought to result from a developmental defect of the intercalated duct system. Surgery may be indicated for diagnosis or for cosmetic reasons (summary by Smyth et al., 1993; Layfield and Gopez, 2002; Eley et al., 2011).
Seifert et al. (1981) first delineated dysgenetic (primary) polycystic disease of the parotid glands as a distinct entity. The disorder was found in 2 unrelated patients out of a series 5,739 cases of salivary gland disturbances, 360 of ... Seifert et al. (1981) first delineated dysgenetic (primary) polycystic disease of the parotid glands as a distinct entity. The disorder was found in 2 unrelated patients out of a series 5,739 cases of salivary gland disturbances, 360 of which were nontumorous cysts of the salivary gland. A 6-year-old girl had painless, recurrently swollen parotid glands bilaterally, although palpation revealed tenderness. Biopsy showed that the gland lobules were permeated with variably sized cysts lined with flattened or cuboidal epithelial cells, some of which showed degenerative changes. Some of the cysts showed spur-like or incomplete septa. There were multiple spheroliths with concentric and radial patterns in the lumens of the cysts. Rests of glandular parenchyma were present between the cysts. The girl's father reportedly had similar changes in the parotid gland until age 12 years. The other patient was a 65-year-old woman with a 9-year history of progressive swelling of the right parotid gland. Sialography showed extended spherical ectasias in the vicinity of the peripheral duct segments. Pathology showed that the gland lobules were interspersed with duct cysts bound by flat epithelium and containing flaky lumps of secretory material. Remnants of gland acini could be seen among the cysts. Seifert et al. (1981) suggested that the cystic changes resulted from a developmental abnormality of the intercalated duct system of the gland, particularly a disturbance of branching and canalization of the terminal salivary duct buds. The differential diagnosis includes congenital sialectasias of the parotid gland, salivary duct cysts, and lymphoepithelial cysts. Dobson and Ellis (1987) reported a 23-year-old woman with bilateral polycystic disease of the parotid gland. She had a history of fluctuating swelling since childhood. Sialogram showed fine punctate mottling of the gland with normal Stensen ducts. Secretory products within the cysts showed congophilic deposits, consistent with amyloid. Batsakis et al. (1988) described the histologic findings in 3 additional unrelated women with the disorder. Two had bilateral parotid swelling since childhood, whereas 1 had unilateral swelling in 1 gland since puberty and developed involvement of the other gland as a young adult. The microscopic changes were virtually identical in all 3 cases: the functional acinar parenchyma of the glands was nearly completely replaced by honeycombed, latticework-like cystic changes. Small clusters of functional acini and ducts appeared as islands amid variably sized cysts. The cystic spaces were lined by flattened or cuboidal epithelial cells. Some of the cells showed hydropic changes and were sloughed into the cysts. In a review of the disorder, Batsakis et al. (1988) noted that secretory products within the cysts can range from a watery fluid to inspissated proteinaceous secretions, which may form laminated microliths or spheroliths. Dysplastic and inflammatory changes are absent, although there may be a mild inflammatory reaction to extravasated fluid. Smyth et al. (1993) reported a mother and daughter with the condition. The daughter presented at age 18 years with bilateral, preauricular, painless swellings. The right-sided swelling began about 2 years prior to presentation, whereas the left-sided swelling had been present since early childhood. Imaging showed multiple cysts confined to the parotid glands. Parotidectomy and subsequent histologic examination showed that most of the gland was replaced by a gelatinous material surrounding multiple cystic spaces containing a mucinous fluid. There were numerous cysts of variable sizes, most lined by low simple squamous epithelium. Cysts were empty or contained inspissated material, mostly amorphous, but sometimes the material was keratin-like material or contained dystrophic calcified foci. The mother had a similar history of progressive swelling of the left parotid gland that began in adolescence; she had had 4 separate operations. There was no history of right-sided facial swelling in the mother. Brown et al. (1995) reported a 31-year-old pregnant woman who presented at 4 months of pregnancy with bilateral painless swelling of the parotid glands with no other features. She had a history of recurrent episodes of swollen parotids as a child. MRI after the birth showed well-circumscribed glands with multiple cysts. Sialogram showed stretching of the intraglandular ductal system with no evidence of sialectasis or duct irregularity. Biopsy showed numerous small cysts lined with flattened to cuboidal epithelial cells, many of which were vacuolated or sloughed into the cyst lumen. Residual normal-appearing acini were scattered among the cysts. A needle aspirate showed an amyloid-like material, which was shown on biopsy to represent eosinophilic spheroliths. Layfield and Gopez (2002) reported a 21-year-old man with the condition. Fine needle aspiration showed small numbers of epithelial cell clusters with a polygonal shape. Most had low columnar to cuboidal appearance with basely situated nuclei; some of the cells contained vacuoles. Parotidectomy showed near total replacement of the acinar tissue with a honeycomb of cystic spaces of various sizes and shapes. The cysts were lined by flattened to cuboidal epithelial cells. Some of the cysts contained multilaminated spheroliths. His father reportedly had the same condition. Layfield and Gopez (2002) discussed the differential diagnosis and cytologic features of various cystic lesions of the parotid gland. Eley et al. (2011) reported an 8-year-old boy with recurrent nontender parotid swelling. He had a history of atopy with asthma, eczema, hay fever, and a nut allergy. Biopsy showed cystic dilation of the terminal ducts throughout the tissue, consistent with PDDP. The authors emphasized the benign nature of the condition.