Ciliated Hepatic Foregut Cyst: Four Case Reports with a Review of the Literature

Fujita, Ayako W.; Steelman, Charlotte K.; Abramowsky, Carlos R.; Ricketts, Richard R.; Durham, Megan M.; Clifton, Matthew S.; Pandya, Samir; Shehata, Bahig M.

doi:10.2350/10-08-0886-cr.1

Cited by 6 publications

(2 citation statements)

References 15 publications

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“…In spite of the CEA levels being elevated (190 ng/mL), the cytology enabled the diagnosis of pancreatic ciliated foregut cysts.Congenital foregut cysts have been diagnosed by EUS-FNA in the mediastinum (most common), liver, and other sites along the hepatobiliary system. 3 The cysts are usually lined by a layer of pseudostratified ciliated epithelium with occasional goblet cells. Foregut cysts arising in relation to the liver and pancreas are hypothesized to originate from a detached outpouching of the primitive foregut, sequestered by the liver and pancreatic tissue during embryological development.…”

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Ciliated foregut cyst of the pancreas: A benign lesion with elevated CEA levels

Bellevicine

Vigliar

Pisapia

et al. 2014

Diagnostic Cytopathology

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Pancreatic cystic lesions represent a challenge for the practicing cytopathologist. Ciliated foregut cysts are congenital cysts arising in relation to the development of the gut and the tracheobronchial tree, and they may occur in a number of sites, most commonly in the mediastinum. 1,2 This rare type of congenital benign cyst may also occur in the subdiaphragmatic region, most frequently in the liver. 3 Conversely, pancreatic ciliated foregut cysts are very uncommon. 1,4,5 The cytopathologist must be aware of their occurrence to avoid overdiagnosis of mucinous neoplastic cysts. Noteworthy, pancreatic foregut ciliated cysts frequently feature elevated intracystic carcinoembryonic antigen (CEA) levels, leading to a worrisome clinical presentation. 6 In this setting, the cytopathologist's role is crucial. The case of a ciliated foregut cyst of the pancreas is illustrated here that was recently encountered in our endoscopic ultrasound (EUS)-pancreatic fine needle aspiration (FNA) practice.In a 50-year-old female, a cystic pancreatic lesion was incidentally discovered by computerized tomography, and it was further investigated by EUS. The cyst was located in the pancreatic head and measured 19 3 9 mm (Fig. 1a). The FNA, performed under EUS guidance, yielded only a few drops of fluid. In our institution, all pancreatic cystic FNAs are routinely performed according to a reference protocol wherein a cytopathologist prepares on site an ethanol-fixed smear for Papanicolaou staining and aliquots the residual material to measure the CEA levels.Microscopically, in a background rich in contaminating normal duodenal cells, loosely cohesive clusters of columnar cells were evident (Fig. 1b). At a higher magnification, these columnar cells displayed a ciliated apical pole and basal, small, round-to-oval normal nuclei (Fig. 1c). Occasionally, a cytoplasmic mucin vacuole displaced and compressed the nucleus (Fig. 1d). In spite of the CEA levels being elevated (190 ng/mL), the cytology enabled the diagnosis of pancreatic ciliated foregut cysts.Congenital foregut cysts have been diagnosed by EUS-FNA in the mediastinum (most common), liver, and other sites along the hepatobiliary system. 3 The cysts are usually lined by a layer of pseudostratified ciliated epithelium with occasional goblet cells. Foregut cysts arising in relation to the liver and pancreas are hypothesized to originate from a detached outpouching of the primitive foregut, sequestered by the liver and pancreatic tissue during embryological development. 7 Cases diagnosed in the pancreas are rare, being frequently misdiagnosed as pancreatic mucinous cystic neoplasms due to the elevated CEA levels. 6 The standard for pancreatic cyst management includes both cytology and CEA level analysis. In fact, the latter can discriminate between cysts that are mucinous or nonmucinous, but only cytomorphology can differentiate between benign and malignant cysts. Unfortunately, a consensus on the cut-off of intracystic CEA levels is still lacking. The majority of studies have shown th...

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confidence: 99%

“…1,2 This rare type of congenital benign cyst may also occur in the subdiaphragmatic region, most frequently in the liver. 3 Conversely, pancreatic ciliated foregut cysts are very uncommon. 1,4,5 The cytopathologist must be aware of their occurrence to avoid overdiagnosis of mucinous neoplastic cysts.…”

mentioning

confidence: 99%