Foregut cysts are uncommon, mucosa-lined congenital lesions that may occur anywhere along the gastrointestinal or respiratory tract and typically present within the first year of life. Although infrequent, these cysts may generate feeding or respiratory difficulties depending on the size and location of the lesion. Foregut cysts of the oral cavity are rarely seen and of those cases localized to the tongue are even more uncommon. We describe a 4-month-old girl with a foregut cyst involving the floor of mouth and anterior tongue. Subsequent histologic analysis demonstrated a cyst lined with both gastric and respiratory epithelia. This case represents an extremely rare finding of both gastric and respiratory epithelia lined within a single cystic structure in the tongue. Although a very rare finding, a foregut cyst should be on the differential diagnosis of any lesion involving the floor of mouth or tongue in an infant or child.
Cysts of foregut origin may localize anywhere along the gastrointestinal tract from the oral cavity to the anus although they are most commonly found in the ileum [
Reported cases of intraoral cysts involving gastric and respiratory epithelia.
Author (year) | Sex | Age | Location | Presentation | Notes |
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Shioda et al. (1971) [ |
— | — | FOM | — | |
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Brown and Kerr-Wilson (1978) [ |
M | 11 mo. | Ventral surface of tongue | Asymptomatic | |
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Tschen (1978) [ |
M | 2 yr. | FOM | Asymptomatic | |
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Mirchandani et al. (1989) [ |
M | 6 mo. | Ventral surface of tongue | Asymptomatic | |
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Mir et al. (1992) [ |
M | 5 mo. | Left FOM + dorsum of base of tongue | Asymptomatic | 2 cysts in patient |
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Ohbayashi et al. (1997) [ |
M | 11 mo. | Dorsum of tongue | Asymptomatic | |
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Said-Al-Naief et al. (1999) [ |
F |
2 yr. |
Anterior tongue |
Difficulty in eating and speech |
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Mandell et al. (2002) [ |
F | 14 days | Ventral surface of tongue | Asymptomatic | |
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Noorchashm et al. (2004) [ |
— | Neonate | Anterior FOM | Asymptomatic | Featuring an intraosseous component through mandible |
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Hall et al. (2005) [ |
M | 13 days | Base of tongue + FOM | Asymptomatic | |
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Leung et al. (2007) [ |
F | 2 days | FOM | Difficulty in feeding | |
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Agaimy et al. (2007) [ |
M | 41 yr. | FOM | Dysphagia, dysphonia | Present at birth; recent malignancy |
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Hartnick et al. (2009) [ |
F | 10 days | FOM | Obstruction of larynx | |
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Houshmand et al. (2011) [ |
F |
2 days |
Anterior tongue |
Asymptomatic |
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Patel et al. (2011) [ |
M | 45 yr. | Anterior tongue | Dysphagia, severe pain, and impaired speech | Present since childhood; recent malignancy |
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Blanchard et al. (2012) [ |
M |
Neonate |
Anterior tongue |
Asymptomatic |
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Joshi et al. (2013) [ |
M | 28 mo. | Ventral surface of tongue | Initially asymptomatic, possible mechanical effect delayed speech | |
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Pentenero et al. (2013) [ |
M | 15 yr. | Anterior tongue + FOM | Progressive dysphagia, dysphonia | Slow growth over 5 years |
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Gantwerker et al. (2014) [ |
F | Neonate | Anterior tongue | Respiratory difficulty | |
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Present case | F | 4 mo. | Ventral surface of tongue + FOM | Asymptomatic |
FOM: floor of mouth.
An infant girl presented with a cystic mass on the floor of the mouth at 6 weeks of age. The slowly growing mass which was present at birth measured approximately 2 cm in diameter on initial examination. At the time of presentation, the patient weighed 4.075 kg, corresponding to the 11th percentile for her age. However, the patient showed no distress and the parents did not report any feeding or breathing difficulties. The family history was unremarkable and there was no history of any other anomalies. Two months later, a magnetic resonance imaging (MRI) was obtained and demonstrated a 1.8 × 2.0 × 1.9 cm T2 hyperintense and mildly T1 hyperintense nonenhancing cystic mass localized to the tongue and sublingual space in the midline with extension to the left of the midline. No fluid-fluid levels were noted. There was no indication that the cyst was arising from the sublingual or submandibular glands and no evidence of extension into the submandibular space (Figure
MRI. (a) T1-weighted, sagittal view: nonenhancing cystic mass in the sublingual space (arrow) without involvement of sublingual or submandibular glands. (b) T2-weighted, axial view: hyperintense mass midline in the anterior lower oral cavity (arrow).
Three weeks after the imaging study was performed, the cystic mass was resected in its entirety using bipolar electrocautery. The cyst was not opened during the procedure. Gross examination of the mass showed a cystic structure measuring 2.5 × 2.1 × 1.8 cm. Sectioning revealed a unilocular cyst measuring 2.5 cm at the greatest dimension which was filled with opaque gelatinous material. Microscopic sections showed a cyst which is lined by respiratory-type ciliated pseudostratified columnar epithelium punctuated at multiple sites by short segments of gastric epithelium. The cyst wall was composed of a continuous layer of skeletal muscle and, to a lesser extent, fibroconnective tissue. Foci of acute and chronic inflammation were present. Several seromucous glands were embedded in the skeletal muscle and fibroconnective tissue. The lumen was filled with mucinous material (Figure
Histology. (a) Transition from respiratory-type ciliated pseudostratified columnar epithelium to gastric type epithelium (arrow). Hematoxylin and eosin, magnification at 400x. (b) The predominant epithelium of the cyst lining is respiratory-type ciliated pseudostratified columnar epithelium. Note the abundance of skeletal muscle with obvious cross striations in the cyst wall (arrows). Seromucinous glands are also present in the cyst wall (bottom right). Hematoxylin and eosin, magnification at 200x. (c) Alcian blue stain highlights the gastric epithelium while the adjacent respiratory-type epithelium is for the most part negative. Magnification at 200x.
The pathogenesis of foregut cysts remains unclear owing to the variability of mucosal linings and locations across the reported cases. There is a general consensus that the often antenatal, neonatal, or infantile presentation of lingual cysts points to some early aberrant embryological event(s) leading to ectopic multipotent cells as the basis for cyst development. Even in the exceedingly rare adult cases, the examination and treatment typically arise from rapid recent changes in cysts observable since infancy or childhood [
In 1970, Gorlin and Jirasek proposed that the heterotopic gastric mucosa seen in oral cysts may be the result of entrapped epithelium of the primitive stomach or stomatodeum [
While a number of theories have been presented over the years, those proposed by Veeneklaas and Gorlin and Jirasek remain the most prominent. In any case, all explanations proposed thus far have failed to incorporate all locations and cell types described in the literature. These differences are readily apparent as the structural anomalies observed by Veeneklass and associated with intestinal foregut cysts, or any other abnormalities for that matter, are notably absent in most reports of lingual foregut cysts. Indeed, the variety of presentations suggests a number of different, albeit possibly similar, causes leading to the manifestation of the various clinical entities classified as foregut cysts or choristomas. It may therefore be impossible to reconcile the development of all cysts under a single theory.
A variety of designations have been used to refer to these cysts in the literature including “lingual cyst,” “enteric duplication cyst,” “foregut cyst,” “lingual choristoma,” “gastrointestinal cyst of the tongue,” and “lingual alimentary cyst,” as well as combinations of the aforementioned terms. Given the confusion surrounding the nomenclature, the use of histologic descriptive terms endorsed by Manor et al. should be favored until these pathologies are better understood or distinguished [
Review of reports of lingual cysts involving both gastric and respiratory epithelia suggests a male predilection (Table
Patients are usually asymptomatic at the time of identification. Among the roughly one-third of cases reporting symptoms, at least half involved some period of observation following discovery and prior to presentation with complaints. Feeding and respiratory difficulties are usually the greatest cause of concern for lingual foregut cysts given the very young age of most patients at the time of presentation but the obstruction or mass effect produced by a cyst can also result in dysphagia, dysphonia, and pain or discomfort [
CT or MRI is often useful in defining the dimensions and anatomic involvement of a cyst and assists in preoperative planning for excision but the definitive diagnosis usually cannot be confirmed until the histopathologic analysis of the specimen. Surgery should be considered, even in the absence of symptoms or complaints, as evolution to malignancy in adults has been reported in previously untreated cysts [
This work was presented as a poster at the Society for Ear, Nose, and Throat Advances in Children (SENTAC) Annual Meeting in St. Louis, MO, on December 5, 2014.
The authors declare that there is no conflict of interests regarding the publication of this paper.
The authors would like to express their thanks to Dr. Priya Sharma for her assistance with interpretation of the radiographic images selected.