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Salivary glands

Epithelial / myoepithelial tumors

Large cell neuroendocrine carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 27 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Extremely rare high grade tumor with neuroendocrine features (organoid, solid, trabecular and rosette-like patterns) and large polygonal cells with coarse chromatin and prominent nucleoli, high mitotic rate, frequent necrosis
● Less than 20 reported cases - must rule out metastatic disease

Clinical features
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● Usually age 60+ years, affects parotid gland
● Aggressive behavior

Case reports
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● 72 and 73 year old men with parotid tumors (Mod Pathol 2000;13:554)
● 74 year old man with parotid mass (Auris Nasus Larynx 2005;32:89)

Treatment
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● Surgery, radiotherapy

Micro description
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● Organoid, solid, trabecular and rosette-like patterns of large polygonal cells with coarse chromatin and prominent nucleoli, high mitotic rate, frequent necrosis

Micro images
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Solid growth of large tumor cells with prominent nucleoli


Organoid growth, peripheral palisading and rosette-like pattern


Neuroendocrine stains and p53

Cytology description
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● Resembles undifferentiatiated carcinoma

Cytology images
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Large, loosely cohesive, pleomorphic tumor cells with prominent nucleoli

Positive stains
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● Neuroendocrine markers (chromogranin, synaptophysin), cytokeratin, p53, BCL2, epidermal growth factor receptor, cyclin D1, Ki-67, CK20

Negative stains
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● Polyoma virus (Am J Surg Pathol 2011;35:1806)

Molecular / cytogenetics description
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● 9p21 LOH and p16 mutation, TP53 LOH and mutation

Electron microscopy images
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Desmosome-like junctions and neurosecretory granules

Differential diagnosis
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Merkel cell carcinoma: CK20+
● Undifferentiated carcinoma

End of Salivary glands > Epithelial / myoepithelial tumors > Large cell neuroendocrine carcinoma


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