Lymphoepithelial Cyst of the Pancreas

Definition

• Pancreatic cystic lesion lined by squamous epithelium with surrounding lymphoid stroma

Diagnostic Criteria

• Unilocular or multilocular lesion
  • Generally filled with keratinous debris
  • May protrude from pancreatic surface
• Cysts lined by mature keratininzing squamous epithelium
  • Occasional cuboidal or transitional appearing areas
  • Rarely focal goblet or sebaceous cells
  • No skin adnexae
• Uniform, dense surrounding lymphoid tissue
  • Frequently with germinal centers
  • May have peripheral space suggestive of subcapsular sinus
  • No acute inflammation
  • Occasional cases with lymphoepithelial-like lesions in surrounding lymphoid tissue
• Surrounding pancreatic tissue normal or focally atrophic

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting : 1/4/08
Updates : 4/26/09, 11/18/12

Supplemental studies

Immunohistology

Differential Diagnosis

• Dermoid cyst
• Epidermoid cyst in intrapancreatic accessory spleen
• Squamoid cyst of pancreatic ducts
• Pancreatic pseudocyst

Most other cysts are easily distinguished by their non-squamous lining

Lymphoepithelial Cyst of the Pancreas	Dermoid Cyst
No skin appendages or mesenchyme	Skin appendages and mesenchyme common
Sebaceous differentiation focal at most	Sebaceous differentiation may be prominent

Lymphoepithelial Cyst of the Pancreas	Epidermoid Cyst in Intrapancreatic Accessory Spleen
Surrounding dense lymphoid tissue	Surrounding splenic red pulp

Lymphoepithelial Cyst of the Pancreas	Squamoid Cyst of Pancreatic Ducts
Surrounding dense lymphoid tissue	No surrounding lymphoid tissue
Overt keratinization	Non-keratinized
Usually peripancreatic	Intraductal

Lymphoepithelial Cyst of the Pancreas	Pancreatic Pseudocyst
Squamous lining (may be partially denuded)	No epithelial lining
Uniform dense lymphoid stroma	Variable surrounding inflammation

Clinical

• Predominantly reported in adults
  • Age range 3-82
• M:F ratio 4:1
• Not associated with HIV, autoimmune disorders or lymphoma
  • Only one reported case associated with HIV (Bedat)
• Benign

Lists

Cystic Pancreatic Lesions

• Acinar cell cystadenocarcinoma
• Acinar cell cystadenoma
• Enterogenous duplication cyst
• Epidermoid cyst in intrapancreatic accessory spleen
• Intraductal oncocytic papillary neoplasm
• Intraductal papillary mucinous neoplasm
• Islet cell / well differentiated neuroendocrine tumor (occasionally cystic)
• Lymphangioma
• Lymphoepithelial cyst
• Mucinous cystic neoplasm
• Paraduodenal wall cyst
• Pseudocyst
• Serous cystadenocarcinoma
• Serous oligocystic adenoma
• Serous microcystic adenoma
• Solid and cystic hamartoma
• Solid pseudopapillary neoplasm
• Squamoid cyst of pancreatic ducts
• von Hippel Lindau associated cystic lesions

Bibliography

• Adsay NV, Hasteh F, Cheng JD, Bejarano PA, Lauwers GY, Batts KP, Kloppel G, Klimstra DS. Lymphoepithelial cysts of the pancreas: a report of 12 cases and a review of the literature. Mod Pathol. 2002 May;15(5):492-501.
• Basturk O, Coban I, Adsay NV. Pancreatic cysts: pathologic classification, differential diagnosis, and clinical implications. Arch Pathol Lab Med. 2009 Mar;133(3):423-38
• Bédat B, Genevay M, Dumonceau JM, Frossard JL, Forget J, Morel P, Berney T. Association between lymphoepithelial cysts of the pancreas and HIV infection. Pancreatology. 2012 Jan-Feb;12(1):61-4
  
• England GM, Allen PW. Selected case from the Arkadi M. Rywlin International Pathology Slide Seminar: benign lymphoepithelial cyst, head of pancreas. Adv Anat Pathol. 2011 Jan;18(1):98-100
  
• Raval JS, Zeh HJ, Moser AJ, Lee KK, Sanders MK, Navina S, Kuan SF, Krasinskas
  AM. Pancreatic lymphoepithelial cysts express CEA and can contain mucous cells: potential pitfalls in the preoperative diagnosis. Mod Pathol. 2010 Nov;23(11):1467-76