Salivary glands

Nonneoplastic tumors/tumor-like conditions

Lymphoepithelial cyst


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Deputy Editor-in-Chief: Kelly Magliocca, D.D.S., M.P.H.
Zahra Maleki, M.D.

Last author update: 12 August 2022
Last staff update: 12 August 2022

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PubMed Search: Benign lymphoepithelial cysts salivary

Zahra Maleki, M.D.
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Cite this page: Maleki Z. Lymphoepithelial cyst. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/salivaryglandsb9lymphoepithelialcyst.html. Accessed August 17th, 2022.
Definition / general
Essential features
Terminology
ICD coding
  • ICD-10: K11.8 - other diseases of salivary glands
Epidemiology
Sites
  • Almost all arise in parotid gland
  • Very rare in submandibular gland
Pathophysiology
  • Sporadic lymphoepithelial cyst may result from cystic dilation of ducts within intraparotid or periparotid lymph node or branchial cleft remnants
  • HIV associated lymphoepithelial cyst likely forms due to hyperplasia of intra-salivary gland lymph nodes and associated ductal obstruction (J Int Assoc Provid AIDS Care 2017;16:120)
  • Sjögren syndrome associated lymphoepithelial cysts arise secondary to infiltration of B cells into the ductal epithelium and their expansion within the striated ducts and subsequent basal ductal cell hyperplasia (Nat Rev Rheumatol 2021;17:333)
Etiology
  • Cystic dilation of the salivary gland ducts
  • HIV associated
  • Autoimmune disease (e.g., Sjögren syndrome) associated
Clinical features
  • Presents as a painless unilocular mass near or within the salivary gland
  • Sporadic and autoimmune related cysts are generally unilateral
  • HIV associated cysts can be bilateral, with an overall incidence of 3 - 5% in HIV patients
Diagnosis
  • Unilateral, painless cystic salivary gland mass
  • Unilateral or bilateral painless cystic salivary gland mass in HIV patients, with or without cervical lymphadenopathy
Radiology description
Radiology images

Contributed by Michael Kraut, M.D.
Lymphoepithelial cyst on coronal section

Lymphoepithelial cyst on coronal section

Lymphoepithelial cyst on axial section

Lymphoepithelial cyst on axial section



Images hosted on other servers:

Well defined mass

Prognostic factors
Case reports
  • 32 year old woman presented with painless swelling of the left side of the neck for 8 months (J Pharm Bioallied Sci 2014;6:S185)
  • 35 year old man with a soft, nontender swelling of the left parotid gland for 9 - 10 months (J Oral Maxillofac Pathol 2018;22:S91)
  • 37 year old woman with a history of HIV and Hodgkin lymphoma presented with right sided facial swelling for 4 days; 47 year old man with a history of HIV / AIDS and Mycobacterium avium complex (MAC) infection presented with right sided jaw mass for 2 months (J Int Assoc Provid AIDS Care 2017;16:120)
Treatment
  • Conservative therapy, with institution of highly active antiretroviral therapy medication in HIV related cases (HIV AIDS (Auckl) 2012;4:81)
  • Surgical treatment not indicated for HIV associated lymphoepithelial cysts unless there is doubt about the diagnosis or there are cosmetic considerations (Head Neck 2018;40:1073)
  • Repeated fine needle aspiration and drainage, sclerotherapy, radiotherapy, surgery (Head Neck 2018;40:1073)
Clinical images

Images hosted on other servers:

Diffuse swelling involving the left parotid gland

Gross description
  • Cystic structure containing a serous clear watery straw colored fluid with smooth and glistening inner lining
Gross images

Images hosted on other servers:

Unilocular cyst with straw colored fluid

Frozen section description
  • Benign lymphoepithelial cyst
Microscopic (histologic) description
  • Most cases show a unilocular cyst with a thin stratified squamous lining
  • Ciliated, cuboidal or columnar epithelial lining is seen in rare cases
  • Epithelium is surrounded by dense polymorphous lymphoid tissue with germinal centers and sinusoidal spaces
  • Lymphocytes frequently permeate the epithelial cyst lining cells
Microscopic (histologic) images

Contributed by Zahra Maleki, M.D.
Lymphoepithelial cyst low magnification

Epithelial and lymphoid component

Lymphoepithelial cyst low magnification

Squamous lining and lymphoid tissue

Lymphoepithelial cyst high magnification

Epithelium surrounded by inflammatory cells

Lymphoepithelial cyst high magnification

Epithelial and inflammatory cells

Cytology description
  • Mature nucleated squamous cells with variable reactive atypia, anuclear cells and squamous epithelium (Int J Surg Case Rep 2017;41:383, Diagn Cytopathol 2012;40:684)
  • Polymorphous lymphocytes and aggregates of epithelioid histiocytes
  • Proteinaceous background
  • Variable presence of acute inflammation, and bland appearing mucinous ductal cells and ciliated columnar cells
  • Scant cellularity, abundant proteinaceous background, lack of squamous cells and epithelial cells, marked atypia of epithelial cells, abundance of lymphocytes pose diagnostic challenges
Cytology images

Contributed by Zahra Maleki, M.D.
Numerous lymphocytes and histiocytes

Numerous lymphocytes and histiocytes

Large epithelial fragment Large epithelial fragment

Large epithelial fragment

Dispersed squamous cells

Dispersed squamous cells

Polymorphous lymphocytes

Polymorphous lymphocytes

Cyst content

Cyst content

Positive stains
Sample pathology report
  • Parotid mass, right, resection:
    • Benign lymphoepithelial cyst with reactive follicular hyperplasia
Sample cytopathology report
  • Parotid, left, fine needle aspiration:
    • Fragments of squamous epithelium, lymphocytes and cyst content (see comment)
    • Comment: The differential diagnosis includes benign lymphoepithelial cyst, cystic lymphoid hyperplasia, lymphoepithelial sialadenitis, benign cyst with lymph node sampling or a Warthin tumor.
    • Recommend: Clinical and radiographic correlation.
Differential diagnosis
Differential diagnosis in cytology
Additional references
Board review style question #1

A cystic parotid mass measuring 1.5 cm is resected. The lesion can be associated with which of the following?

  1. EBV associated
  2. HHV8 associated
  3. HIV associated
  4. HPV associated
Board review style answer #1
C. HIV associated

Comment Here

Reference: Lymphoepithelial cyst
Board review style question #2

FNA of a cystic parotid mass in an 11 year old child is shown here. The most likely diagnosis is

  1. Granulomatous inflammation
  2. Lymphoepithelial cyst
  3. Mucoepidermoid carcinoma
  4. Squamous cell carcinoma
Board review style answer #2
B. Lymphoepithelial cyst

Comment Here

Reference: Lymphoepithelial cyst
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