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7 Quick Facts about Sclerosing Polycystic Adenosis

 

Dr Sampurna Roy MD   

 

   

Sclerosing polycystic adenosis (SPA) is a recently described pseudoneoplastic salivary gland lesion of unknown etiology. Only 51 cases have been described in the English-language literature. 

Here are 7 quick facts about this exceedingly rare salivary gland lesion.

 

 

 

                         

      

Microscopic examination reveals lobular proliferation of dilated ductal components with cystic changes surrounded by abundant dense, hyalinized, hypocellular, collagen stroma.

Inset: The dilated ducts are lined with flatted cuboidal cells with foci of apocrine metaplasia.

 

 

Source: Park I-H, Hong S-M, Choi H, Chang H, Lee H-M. Sclerosing Polycystic Adenosis of the Nasal Septum: The Risk of Misdiagnosis. Clinical and Experimental Otorhinolaryngology. 2013;6(2):107-109

 

 

(1) In 1996, Smith et al first described sclerosing polycystic adenosis in a series of 9 cases.

 

(2) It shows morphologic similarity to fibrocystic disease/sclerosing adenosis and intraductal epithelial proliferations of various types of the breast.

 

(3) About 80% of SPA cases present in the major salivary glands, specifically, the parotid gland. However, cases have been reported in the minor salivary glands of the nasal septum, buccal mucosa, hard palate, floor of the mouth, retromolar pad and in the lacrimal gland.

 

(4) Microscopic features:

Sclerosing polycystic adenosis is well-circumscribed, unencapsulated, and composed of lobules of proliferating ductules with occasional apocrine metaplasia, ductal hyperplasia, and focal cystic spaces within a dense collagenous hypocellular stroma.

The glandular epithelial cells exhibit apocrine, foamy vaculolated and mucinous cells.

The acinar cells often contain prominent eosinophilic material consistent with altered zymogen granules.

The ductal epithelial atypia, ranging from mild dysplasia to carcinoma in situ have been reported in some cases.

 

 

 

                         

             

Immunohistochemical staining for smooth muscle actin demonstrate myoepithelial cells surrounding ductal epithelium. Focal ductal epithelial cells show immunoreactivity for estrogen receptor.

 

 

Source: Park I-H, Hong S-M, Choi H, Chang H, Lee H-M. Sclerosing Polycystic Adenosis of the Nasal Septum: The Risk of Misdiagnosis. Clinical and Experimental Otorhinolaryngology. 2013;6(2):107-109

 

 

(5) Differential diagnosis include pleomorphic adenoma; benign polycystic disease; sclerosing sialadenitis; and malignant glandular neoplasias, such as mucoepidermoid carcinoma, acinic cell carcinoma, adenocarcinoma NOS (not otherwise specified), and salivary duct carcinoma.

 

(6) Although atypia ranging from mild dysplasia to carcinoma in situ can occur in some cases, SPA has a favorable outcome. In SPA, the lobular architecture is typically maintained, the atypical nests are rimmed by myoepithelial cells, and the invasive, destructive growth pattern of a carcinoma is lacking. If clinicians and pathologists are not aware of this condition, there is high chance of misdiagnosis.

 

(7) Treatment for SPA is surgical excision. Recurrence occurs in almost one-third of cases probably due to incomplete surgical excision or multifocal disease.

 

Further reading:

 

Sclerosing Polycystic Adenosis of the Nasal Septum: The Risk of Misdiagnosis. Clinical and Experimental Otorhinolaryngology. 2013;6(2):107-109

 

Sclerosing Polycystic Adenosis of Salivary Glands: A Review with Some Emphasis on Intraductal Epithelial Proliferations

 

Sclerosing Polycystic Adenosis of the Buccal Mucosa

 

Sclerosing Polycystic Adenosis: A Rare Tumor of the Salivary Glands

 

 

 

 

 


 

 

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