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Salivary glands

Epithelial / myoepithelial tumors

Small cell 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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● Rare aggressive tumor resembling small cell carcinoma of lung (Am J Surg Pathol 2004;28:762)
● Pure or with squamous cell carcinoma or adenocarcinoma

Clinical features
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● 2% of parotid gland carcinomas, 4% of minor salivary gland malignancies
● Usually age 50+ but also < 30 years
● Either neuroendocrine (Merkel cell or pulmonary varieties) or ductal types (arising from ductal neuroendocrine stem cells, Pathol Int 1999;49:887)
● 2 and 5 year survival of 70% and 46%

Case reports
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● 48 year old woman with 3 year history of right cheek mass (Head Neck Pathol 2012;6:502)
● 61 year old man with parotid tumor (Arch Pathol Lab Med 1995;119:183)
● 63 year old man with oral cavity tumor (Mod Pathol 1990;3:631)
● 79 year old man with submandibular mass (Ear Nose Throat J 2012;91:E10)
● 91 year old man with good outcome after irradiation (Clin Transl Oncol 2008;10:303)

Treatment
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● Chemotherapy, radiotherapy

Micro description
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● Resembles lung tumors with solid areas of small spindled to ovoid cells with minimal cytoplasm, hyperchromatic nuclei with fine chromatin, indistinct nuclei
● High mitotic activity, geographic necrosis
● Often a better differentiated carcinoma is present
● Rarely squamous or ductal differentiation
● Does not arise from surface epithelium but may involve it secondarily
● May occur in carcinoma ex pleomorphic adenoma

Micro images
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Various images


87 year old woman with long term survival

Positive stains
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● Keratin (punctuate perinuclear staining), CK20 (75%, paranuclear dotlike pattern, Am J Surg Pathol 1997;21:226), EMA, at least one neuroendocrine marker (chromogranin, synaptophysin, CD57/Leu7, CD56, neuron specific enolase)

Negative stains
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● TTF1, Merkel cell polyoma virus (Am J Surg Pathol 2011;35:1806)

Electron microscopy description
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● Variable dense core secretory granules

Differential diagnosis
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Adenoid cystic carcinoma: solid variant resembles small cell carcinoma, but has focal cribriform architecture, myoepithelial differentiation, no neuroendocrine differentiation
Large cell neuroendocrine carcinoma: cells > 30 microns, moderate N/C ratio, coarse chromatin, prominent nucleoli
Lymphoma: CD45+, keratin-
Melanoma: S100+, HMB45+, vimentin+, CK-
● Merkel cell carcinoma
Metastases: small cell carcinoma from lung or other site; squamous cell carcinoma
● Small cell anaplastic carcinoma with neuroendocrine features
● Small cell epidermoid carcinoma: different findings on electron microscopy
● Small cell undifferentiated carcinoma: negative neuroendocrine markers

End of Salivary glands > Epithelial / myoepithelial tumors > Small cell carcinoma


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