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Salivary glands

Epithelial / myoepithelial tumors

Signet ring cell adenocarcinoma

Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 1 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Rare adenocarcinoma subtype first described by Ghannoum and Freedman (Am J Surg Pathol 2004;28:29)
● Represents 2% of primary minor salivary gland malignancies; less common in major salivary glands
● Adults, mean age 56 years, female predilection
● Slow growing with favorable outcome; low grade behavior with no recurrence / metastases, based on limited data

Case reports

● 18 year old man (youngest reported, Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110:e33)
● 38 year old woman with parotid mass (Head Neck 2011;33:1656)
● 52 year old man with 40 pack-year smoking history (Diagn Cytopathol 1997;17:374)
● Involvement of lower lip with low grade behavior (Hum Pathol 2012;43:303)

Micro description

● Non-circumscribed tumor composed of bland, extracellular mucin containing signet ring cells (scattered cells or nests) that invade in narrow parallel strands, with scattered small nests or individually infiltrating cells, eccentric indented nuclei, mild atypia; mixed with eosinophilic and clear cells
● Often perineurial invasion, but no angiolymphatic invasion
● Minimal ductal differentiation
● No necrosis, no/rare mitoses,
● No solid, cribriform or papillary components

Micro images

Encapsulated and centrally cystic

Cells have large intracytoplasmic vacuole with Alcian blue-positive mucin

CK14+ basal cells of residual ducts, which contain some signet ring cells simiar to the tumor cells

Positive stains

● CAM 5.2, smooth muscle actin, GFAP, p63, cytokeratin, MUC1, MUC2, MUC5AC, MUC6, GCDFP15

Negative stains

● Calponin, HER2, myoepithelial markers

Differential diagnosis

● Colloid (mucinous) carcinoma
● Mammary analogue secretory carcinoma: see Histopathology 2012;61:387
● Metastatic signet ring cell adenocarcinoma (lung)
Mucoepidermoid carcinoma
Polymorphous low grade adenocarcinoma
Salivary duct carcinoma: see Med Mol Morphol 2012;45:45 for mucin-rich variant

End of Salivary glands > Epithelial / myoepithelial tumors > Signet ring cell adenocarcinoma

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