Sclerosing polycystic adenosis of salivary glands: a review with some emphasis on intraductal epithelial proliferations.

Sclerosing polycystic adenosis (SPA) is a rare condition of salivary glands. The most common site is the parotid gland (80 % of cases). SPA shows no gender predilection and occurs over a wide age spectrum (9-84 years). SPA is mostly unifocal, but may rarely be multifocal. Histologically, SPA are sharply circumscribed mostly unencapsulated lesions composed of acinar and ductal components with variable cytomorphological characteristics, including foamy, vacuolated, apocrine, mucous, clear/ballooned, squamous, columnar and oncocyte-like cells. Characteristic for SPA is the presence of large acinar cells with abundant eosinophilic cytoplasmic granules. The stroma is densely collagenized, frequently harbouring a variably intense chronic inflammatory infiltrate and may contain fat. Rarely the stroma is myxoid. Some degree of intraductal epithelial proliferations have been reported in at least 50 % of cases. The proportion of cases with epithelial proliferations that fulfill criteria for high-grade ductal carcinoma in-situ is <10 %. Immunohistochemically, both ductal and acinar cells are positive for broad spectrum cytokeratins. There is variable immunoreactivity for epithelial membrane antigen and S-100 protein. CEA, p53 and HER2 is reportedly negative. Gross cystic disease fluid protein-15 is strongly expressed in the acinar component. There is consistent but variable expression of estrogen and progesterone receptors. The proliferative index (Ki-67) is low (1-2 %) in the benign (acinar and ductal) components. Using HUMARA methodology (non-random inactivation of X-chromosomes), six cases with atypical epithelial proliferations have been shown to be clonal processes. Recurrences have been reported in up to 19 % of cases.