Mandible-Maxilla
Fissural and other non-odontogenic cysts
Oral foregut duplication cyst

Author: Anthony Martinez, M.D. (see Authors page)
Editor: Kelly R. Magliocca, M.D.

Revised: 5 November 2015, last major update November 2015

Copyright: (c) 2004-2015, PathologyOutlines.com, Inc.

PubMed Search: Oral foregut duplication [title] cyst
Cite this page: Oral foregut duplication cyst. PathologyOutlines.com website. http://pathologyoutlines.com/topic/mandiblemaxillaforegutcyst.html. Accessed March 29th, 2017.
Definition / General
  • Oral cavity cyst that is a choristoma (normal cells or tissues in an abnormal location), most commonly composed of heterotopic gastric mucosa
  • Foregut gives rise to the pharynx, lower respiratory tract and upper gastrointestinal tract (esophagus, stomach, duodenum and hepatobiliary system)
  • Although these cystic lesions are variably named (see Terminology) and have varied cyst linings (gastrointestinal, respiratory, squamous or mixed), it would be difficult to define each as a distinctive entity because the age and mode of presentation is strikingly similar
    • Anatomic level of origin may determine the nature of the lesion
Terminology
  • Oral foregut duplication cyst
  • Lingual cyst
  • Lingual choristoma
  • Enteric duplication cyst
  • Heterotopic gastrointestinal cyst of oral cavity
  • Enterocystomas: enteric duplication cyst of the tongue
Epidemiology
  • Rare cyst of infants and children - fewer than 100 cases reported
  • Most cases discovered by 2 years of age
  • Slight male predominance (3:2)
Sites
  • Can occur anywhere from the mouth to anus
  • In oral cavity, anterior oral tongue is most common site (60% of reported cases)
Etiology
  • Unknown etiology but several theories proposed:
    • Variant of split notochord syndrome
      • Disturbance in the development of the notochord and surrounding structures causes adherent endodermal cells to become caught during the infolding of the notochord plate
      • The endodermal cells give rise to GI type mucosa in cysts
    • Heterotopia or supernumerary buds
      • During the first trimester, heterotopic rests of foregut-derived epithelium may persist, resulting in foregut duplication cysts
      • Gastric mucosa may be derived from entrapped embryonic gastrointestinal epithelium or ectoderm from the primitive stomodeum
    • Entrapped rests: endodermal rests of the stomodeum are trapped by the lateral lingual swellings and subject to inductive influences causing differentiation into gastrointestinal epithelia
Clinical Features
  • Usually presents as asymptomatic swelling in floor of mouth
  • 30% have difficulty in feeding, swallowing, respiration or speech
Diagnosis
  • Combination of clinical, radiologic and histologic findings
Radiology Description
  • Appear as cystic lesions
  • They may be hyperintense on T1 weighted images, depending on the amount and distribution of proteinaceous contents
  • They uniformly appear hyperintense on T2 and short T1 inversion recovery sequences
  • Enteric duplication cysts are often indistinguishable from dermoid cysts on MRI because of the presence of proteinaceous fluid
  • On MRI, foregut duplication cysts can be of variable signal intensity on T1 weighted images, which is related to the protein content of the cyst fluid
  • On T2 weighted images, the cyst is typically bright and without internal septations or soft tissue nodularity
  • The absence of restricted diffusion on diffusion-weighted imaging helps distinguish a foregut duplication cyst from a dermoid cyst
Radiology Images

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MRI sagittal image

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T1 weighted

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MR images

Case Reports
Treatment
  • Usually surgical excision
Clinical Images

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Cystic mass

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Sublingual cyst

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Cystic mass, floor of mouth

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Tongue mass

Gross Images

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Smooth mucosal lining

Micro Description
  • Traditionally, there are 3 criteria that must be met to make a diagnosis of foregut duplication cyst
    • Covered by a smooth muscle layer
    • Contain epithelium derived from foregut
    • Be attached to a portion of foregut
  • Duplication cysts are lined by 1 or more types of epithelium: gastric mucosa, ciliated respiratory type epithelium, stratified squamous epithelium or simple cuboidal epithelium
Micro Images

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Gastric foveolar epithelium

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Respiratory epithelium

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Colonic and gastric mucosa

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Seromucinous glands present

Differential Diagnosis
  • Dermoid cyst
    • Can have a similar appearance on MRI
    • Lined by stratified squamous epithelium with associated hair follicles and sebaceous glands
  • Lingual thyroid
    • Thyroid tissue at base of tongue, between epiglottis and foramen cecum, due to failure to descend to neck
    • 70% lack a normal thyroid gland
  • Thyroglossal duct cyst
    • Develops from cells and tissues remaining after formation of thyroid gland during embryonic development
    • Appears anywhere along path of thyroglossal duct, from foramen cecum in tongue base to suprasternal region
    • Type of epithelial lining varies by site:
      • Lined by squamous epithelium if high in neck (near tongue and foramen cecum)
      • Lined by respiratory epithelium if lower in neck
      • Pseudostratified ciliated epithelium is dominant superior to hyoid bone, perhaps due to its close proximity to upper respiratory tract
      • Stratified cuboidal epithelium is present at level of hyoid bone