Pancreas - Lymphoepithelial cysts

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Pancreas

Cysts

Lymphoepithelial cysts

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 24 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Rare congenital cyst similar to branchial pouch derived structures; 0.5% of pancreatic cysts
● First describedi n 1987 (Am J Surg Pathol 1987;11:899)
● Mean age 56 years, range 35-82 years, 80% male (Mod Pathol 2002;15:492)
● Present with abdominal pain or nausea, or incidental finding
● Not associated with immunosuppression or autoimmune diseases
● Occurs anywhere in pancreas (head, body, or tail) or extrapancreatic

General
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● Etiology may be similar to Warthin’s tumors, in which lymphoid cells have an affinity for ductal epithelia and can induce their growth (Adv Anat Pathol 2011;18:98)
● May occasionally develop from epithelial remnants in lymph nodes (Hum Pathol 1991;22:924) or from accessory spleen in pancreas (Mod Pathol 1998;11:1171)

Clinical features
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● Benign, do not recur or progress
● Elevated CA19-9, CEA levels in cyst fluid (Mod Pathol 2010;23:1467)

Case reports
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● 60 year old man (Case Rep Gastroenterol 2012;6:604)

Gross description
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● Mean 5 cm (range, 1-17 cm), cysts contain keratin or clear fluid
● Often round and well-demarcated from surrounding pancreas, 60% multilocular

Gross images
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Well demarcated cysts

Cyst with papillary projection

Micro description
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● Unilocular or multilocular, lined by squamous epithelium, with lymphocytes and germinal centers in the wall
● Occasional solid lymphoepithelial islands, rarely mucinous goblet cells
● Rare sebaceous differentiation, keratin granulomas may be present

Micro images
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Various images

Cyst with papillary projection of squamous epithelium surrounded by lymphoid tissue

Cytology description
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● Numerous anucleated squamous cells, amorphous debris (Cancer 2006;108:501)
● May have mildly atypical mucinous glandular and parakeratotic epithelium
● Rare benign nucleated cells, usually no lymphocytes

Cytology images
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Well-differentiated squamous epithelium, keratinaceous and amorphous debris, and some lymphoid cells

Squamous cells with no/rare lymphocytes

Various images

Differential diagnosis
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● Clinical differential diagnosis includes mucinous cystic neoplasms, IPMN, IOPN (but different histologically)
● Histological differential diagnosis: dermoid and epidermoid cysts (younger-mean ages 29/37 years, M=F, prominent mucinous cells or respiratory mucosa in dermoid cyst), lymphangioma (positive for endothelial and lymphatic markers), pseudocyst (peripancreatic, no lymphoid stroma)

End of Pancreas > Cysts > Lymphoepithelial cysts

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