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Ciliated foregut cysts involving the hepatopancreaticobiliary system: a clinicopathological evaluation with focus on atypical features
  1. Pooja Navale1,
  2. Jonathan Glickman2,
  3. Imad Nasser3,
  4. Jinru Shia4,
  5. Monika Vyas3
  1. 1Department of Pathology, Washington University in St Louis, St Louis, Missouri, USA
  2. 2Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
  3. 3Department of Pathology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
  4. 4Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  1. Correspondence to Dr Pooja Navale, Pathology, Washington University in St Louis, St Louis, Missouri, USA; navale{at}


Aims Foregut cystic malformations are rare developmental abnormalities, which may involve the hepatopancreaticobiliary tract (HPBT). These cysts are composed of inner ciliated epithelium; subepithelial connective tissue layer; smooth muscle layer; and an outer fibrous layer. While radiopathologic findings are often diagnostic, atypical location and histologic features can pose a diagnostic challenge. We aimed to study ciliated foregut cysts (CFCs) in the HPBT, assess their clinicopathological features with a focus on atypical features.

Methods We collected cases of CFCs involving the HPBT from three large academic medical centres. H&E-stained slides and immunohistochemical stains (where available) were reviewed for each case. Relevant demographic, clinical and pathological information was collected from the medical records.

Results 21 cases were identified. The median age was 53 years (range, 3–78 years). 17 cysts were identified within the liver (segment 4 was the most common location, n=10) and 4 in the pancreas. Cysts were mostly identified incidentally (n=13), abdominal pain was a common symptom (n=5). Cyst size ranged from 0.7 to 17.0 cm (median, 2.5 cm). Radiological findings were available in 17 cases. Cilia were identified in all cases. 19 of 21 cases demonstrated the presence of a smooth muscle layer (thickness, <0.1 mm to 3.0 mm). Three cases showed gastric metaplasia, while one case revealed additional low-grade dysplasia, with features similar to intraductal papillary neoplasm of the bile duct.

Conclusions We highlight clinicopathological features of CFCs in the HPBT. The histomorphology is usually straightforward; however, unusual location and atypical features can pose a diagnostic challenge.

  • Biliary Tract

Data availability statement

All data relevant to the study are included in the article or uploaded as an online supplemental information.

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Data availability statement

All data relevant to the study are included in the article or uploaded as an online supplemental information.

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  • Handling editor Vikram Deshpande.

  • Twitter @navale_pooja

  • Contributors PN and MV contributed to the study conception/design, data collection, data review and manuscript review. JS contributed to data collection and manuscript review. JG and IN contributed to data collection and manuscript review. The first draft of the manuscript was written by PN. All authors have read and approved the final manuscript. PN and MV are responsible for the overall content as the guarantors

  • Funding MSK Cancer Center Support Grant/Core Grant (P30 CA008748).

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.