Sclerosing Polycystic Adenosis

Sclerosing polycystic adenosis is a pseudoneoplastic

inflammatory lesion which usually presents as a discrete
mass in the parotid gland.

It is composed of fibrohyaline stroma containing dilated

ductal and acinar structures as shown here.

It bears some resemblance to sclerosing adenosis and

fibrocystic disease of the breast
Embedded in sclerotic and hyalinized stroma are ducts
and glands, some lined by apocrine cells (the largest
duct in the center is lined by cells with apical snout-like
projections, i.e. apocrine type of secretion). 
Higher magnification showing a combination of
apocrine and mucoid cells with zymogen-like
granules.

The clinical behavior is generally benign.

However, some cases show enough atypia to
consider the diagnosis of a low-grade malignant
neoplasm.

Some studies have shown that the lesion is clonal,

further generating controversy as to its true
nature.
Sclerosing polycystic adenosis shows focal
accumulations of cystically dilated ducts in a sclerotic
fibrous stroma. Areas with a cribriform or papillary pattern
can be seen.
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.
Immunohistochemical staining for smooth muscle
actin demonstrate myoepithelial cells surrounding
ductal epithelium.

Focal ductal epithelial cells show immunoreactivity

for estrogen receptor.
It shows morphologic similarity to fibrocystic disease/sclerosing adenosis and intraductal epithelial
proliferations of various types of the breast.
 
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.
 
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.
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.
 
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.
 
Treatment for SPA is surgical excision. Recurrence occurs in almost one-third of
cases probably due to incomplete surgical excision or multifocal disease.