Pancreas
Cysts
Lymphoepithelial cysts

Editor-in-Chief: Debra Zynger, M.D.
Katrina Krogh, M.D.
Guang-Yu Yang, M.D., Ph.D.

Topic Completed: 4 April 2019

Revised: 4 April 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Lymphoepithelial cysts[TIAB] pancreas

Katrina Krogh, M.D.
Guang-Yu Yang, M.D., Ph.D.
Page views in 2018: 1,089
Page views in 2019 to date: 1,249
Cite this page: Krogh K Lymphoepithelial cysts. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreaslymphoepithelial.html. Accessed October 21st, 2019.
Definition / general
  • Extremely rare, nonneoplastic, true cyst lined by squamous epithelium with abundant lymphoid tissue
  • Histologically similar to branchial cleft cysts arising in the neck
  • First described in 1985 (Pathologe 1985;6:217)
  • No association with salivary gland lymphoepithelial cysts
Essential features
  • Consists of a squamous epithelial-lined cyst with surrounding lymphoid tissue
ICD coding
  • ICD-10: K86.2 - cyst of pancreas
Epidemiology
Sites
  • Occurs anywhere in pancreas (head, body or tail) or extrapancreatic location
Pathophysiology
Etiology
  • Etiology is unclear; still debated whether lymphoepithelial cysts are derived from embryonic remnants or pre-existing tissues with acquired inflammatory change (Medicine (Baltimore) 2015;94:e1758)
  • Another possibility notes similarity to Warthin tumors in which lymphoid cells have an affinity for ductal epithelia and can induce their growth (Adv Anat Pathol 2011;18:98)
Clinical features
  • Presents with nonspecific symptoms such as abdominal pain or nausea; ~50% present incidentally (JOP 2013;14:250)
  • Not associated with immunosuppression or autoimmune diseases
Diagnostic criteria
  • Often diagnosed on fine needle aspiration by cytopathologist
  • Can be difficult to differentiate preoperatively from other pancreatic cystic lesions
Laboratory
  • Cyst fluid has elevated CA19-9 levels and occasionally elevated CEA levels (diagnostic pitfall) (Mod Pathol 2010;23:1467)
Radiology description
  • On CT, enhancement often equivocal; can look similar to serous cystic neoplasms, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms
  • Imaging varies and is similar to other pancreatic lesions (JOP 2013;14:15)
Radiology images

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CT

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Large cystic mass

Prognostic factors
  • Benign, does not recur or progress
Case reports
Treatment
  • Resection is curative
Clinical images

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Intraoperative view

Gross description
  • Mean size 5 cm (range 1 – 17 cm)
  • Often round and well demarcated from surrounding pancreas
  • Either multilocular (60%) or unilocular (40%)
  • Cysts contain serous or cheesy keratin-containing material
Gross images

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Well demarcated cysts

Cyst with papillary projection

Pancreas tail

Microscopic (histologic) description
  • Unilocular or multilocular cyst lined by squamous epithelium (nucleated or anucleated), with lymphocytes (intervening T lymphocytes) and germinal centers in the wall
  • Combinations of squamous lining with other epithelial types (i.e. simple columnar) have been reported
  • Lymphoid tissue, often a dense band
  • With or without keratinous debris
  • Occasional solid lymphoepithelial islands, rarely mucinous goblet cells
  • Rare sebaceous differentiation, keratin granulomas, cholesterol clefts, multinucleated giant cells and foamy histiocytes may be present
Microscopic (histologic) images

Contributed by Katrina Krogh, M.D.

Lymphoepithelial cyst of the pancreas

Cytology description
  • 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

Images hosted on other servers:

Squamous
 epithelium,
keratinaceous debris
and lymphocytes

Squamous cells with rare lymphocytes

Keratinaceous debris

Sample pathology report
  • Pancreas, cyst, excision:
    • Lymphoepithelial cyst
Differential diagnosis
Board review question #1
Which of the following statements is true about the following pancreatic lesion?

  1. This lesion is more common than pancreatic mucinous adenocarcinoma
  2. This lesion does not undergo malignant transformation
  3. This lesion is found in females more commonly
  4. CEA is never elevated in cyst fluid
Board review answer #1
B. This lesion does not undergo malignant transformation

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Board review question #2
Which of the following statements about this pancreatic lesion is true?

  1. This lesion is the most common cystic lesion of the pancreas
  2. This lesion is caused by immunosuppression or autoimmune dysfunction
  3. Foamy histiocytes can be present
  4. This lesion is associated with salivary gland lymphoepithelial cysts
Board review answer #2
C. Foamy histiocytes can be present

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