Salivary glands - Adenocarcinoma, not otherwise specified (NOS)

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Salivary glands

Epithelial / myoepithelial tumors

Adenocarcinoma, not otherwise specified (NOS)

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.

General
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● Invasive tumor, often aggressive, with glandular or ductal differentiation but no features characteristic of other specific types (Arch Pathol Lab Med 2004;128:1385)
● Common, 5-10% of salivary gland tumors
● 6-10% of salivary gland malignancies, 17% of parotid gland malignancies, 15% of minor salivary gland malignancies

Clinical features
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● Mean age 58 years (median 67 years), range 10-93 years
● Usually asymptomatic
● Often fixed to skin or deep tissues
● Palatal lesions often ulcerated and involve bone
● Gender predominance debated, recent reports show male predominance
● Cervical lymph node metastases in 23%, distant metastases in 37%
● Diagnosis of exclusion (not metastatic, not another salivary gland carcinoma)
● 5 year disease specific survivals is 57%

Sites
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● Parotid gland, submandibular gland, palate, buccal mucosa

Case reports
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● 49 year old man with parotid mass (Arch Pathol Lab Med 2004;128:487)

Treatment
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● Complete surgical excision

Gross description
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● Poorly circumscribed with infiltrative borders
● Solid tan cut surface with hemorrhage and necrosis

Micro description
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● Invasive with glandular or ductal differentiation but no features characteristic of other specific types
● Patterns include glandular spaces with cyst formation, papillary formation, solid sheets, comedonecrosis, hyalinized “shadow” nodules
● Small clusters of cuboidal, round or ovoid cells with distinct borders and abundant cytoplasm
● May have clear cell or oncocytic features
● Low, intermediate or high grade based on cytomorphic features
● In situ component in 68% (Virchows Arch 2006;449:159)

Micro images
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Cellular tumor with glandular or ductlike structures, occasional pink cytoplasmic granules, occasional mucicarmine+ cells

Various images

Positive stains
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● CK7+/CK20- (Mod Pathol 2004;17:407), positive for EGFR, survivin, phosphoSTAT3, CK18, HER2
Uniform periductal staining of reactive myofibroblastic cells with calponin, smooth muscle actin, smooth muscle myosin heavy chain (Arch Pathol Lab Med 1999;123:801)

Negative stains
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● MYB (Mod Pathol 2011;24:1169)

Molecular / cytogenetics description
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● EGFR gene amplification, increased EGFR gene copy number
● HER2 amplification, high HER2 gene copy number
● KRAS mutation rarely (Arch Pathol Lab Med 2000;124:836)

Differential diagnosis
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● Hybrid carcinoma
● Membranous adenoma
● Metastatic adenocarcinoma
● Polymorphous low grade adenocarcinoma
● Undifferentiated carcinoma

End of Salivary glands > Epithelial / myoepithelial tumors > Adenocarcinoma, not otherwise specified (NOS)

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