Ciliated foregut cyst in the triangle of Calot: the first report
© Al Beteddini et al. 2016
Received: 27 October 2015
Accepted: 22 February 2016
Published: 25 February 2016
Ciliated foregut cysts are rare anomalies arising from remnants of aberrant embryological development. Around 100 reports on the presence of these congenital masses in the tracheobronchial tree, mediastinum, liver, pancreas and, rarely, the gallbladder have been described. In this article, the case of a 33-year-old woman, who was operated for a laparoscopic cholecystectomy, is presented. During the dissection of the triangle of Calot, a cystic mass, attached to the common hepatic duct, was discovered incidentally. This cyst was dissected off the hepatic duct, and no communication between both structures was found. The histopathological diagnosis was consistent with a ciliated foregut cyst. The postoperative course was uneventful. After reviewing the literature on this pathological entity, we found that this is the first report of a ciliated foregut cyst that is located in the triangle of Calot and found separate from the biliary structures, the gallbladder and the liver. We present a review of the literature on this entity, discussing diagnostic measures and therapeutic options.
The presence of cysts originating from the embryonic primitive foregut and containing a respiratory-type epithelial lining has rarely been described in medical literature. These cysts result from aberrant embryological development and are usually located above the diaphragm. Their presence in relation to abdominal organs has been described in around 100 reports. We present the first report of a ciliated foregut cyst in the triangle of Calot, in relation to the common hepatic duct. The diagnostic and therapeutic implications and the malignant potential of these lesions are further discussed.
Ciliated foregut cysts (CFC) are extremely rare, benign, congenital cystic lesions that arise from the embryonic primitive foregut . These cysts are most often solitary and unilocular characterised by an internal pseudostratified, ciliated, mucin-secreting, columnar epithelial lining . These lesions are usually located above the diaphragm [2, 3] with a few reports of ciliated cysts in relation to abdominal organs.
During embryonic development, the differentiation of the primitive foregut cells gives rise to the oropharynx, oesophagus, larynx, tracheobronchial tree, lungs, stomach, proximal duodenum, pancreas, liver and biliary system [3–5]. The presence of cysts with a respiratory-type epithelial lining in relation to abdominal organs is aberrant. However, it is postulated that the same mechanisms underlie the development of CFC in relation to abdominal organs and the respiratory system [3, 10].
Consequently, the association of CFC with the biliary system is quite exceptional. The first description of these lesions in the gallbladder was by Kakitsubata et al. in . A ciliated cyst of the common bile duct was reported by Baranger et al. in . In 2000, Nam et al. were the first to introduce the term ‘ciliated foregut cyst of the gallbladder’ in their report . Five other reports described ciliated cysts found within the wall of the gallbladder [2–4, 8, 9].
The differential diagnosis of hepatic CFC would include biliary cyst, parasitic cyst, mucinous cystic neoplasm and various cystic metastases, such as cystic neuroendocrine tumour or necrotic metastases. When identified in the gallbladder fossa, a pancreatic pseudocyst, choledochal cyst or gallbladder duplication should also be considered .
To our knowledge, this is the first description of a ciliated foregut cyst in the triangle of Calot presenting as a unilocular cyst that is attached by fibrous tissue to the common hepatic duct and is totally extramural and without any communication to the gallbladder or the hepatic ducts .
This case highlights the diagnostic challenges and management options posed by this pathological rarity. In this case, the pre-operative ultrasound scan could not specifically identify the presence of the cyst, and an intra-operative cholangiogram could not prove a communication between the cyst and the biliary tracts. Furthermore, there were reports on the transformation of hepatic ciliated foregut cysts into primary squamous cell carcinoma [9, 10]. The exceptional presentation of such a lesion precludes firm conclusions in this regard. As malignant potential cannot be totally excluded and in the absence of well-defined surveillance criteria, the excision of these tumours, when diagnosed, would be a rational approach mainly in young and symptomatic patients.
Ciliated foregut cysts in relation to abdominal organs are a pathological rarity. This is the first report of a foregut cyst in the triangle of Calot. Due to this incidental presentation, no firm conclusions can be drawn with regard to the diagnostic workup and management plan. However, sharing histological similarities with the more common hepatic ciliated foregut cysts, which are known to have neoplastic potential, the excision of these tumours would be a rational approach mainly in young and symptomatic patients.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-chief of this journal.
The authors would gratefully acknowledge Dr. Yusra Kadhim (consultant radiologist) for her kind assistance in reading the imaging studies related to the case.
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- Wheeler DA, Edmonson HA. Ciliated hepatic foregut cyst. American J Surgical Pathology. 1984;8:467–70.View ArticleGoogle Scholar
- Muraoka A, Watanabe N, Ikeda Y, Kokudo Y, Tatemoto A, Sone Y, et al. Ciliated foregut cyst of the gallbladder: report of a case. Surg Today. 2003;33(9):718–21.View ArticlePubMedGoogle Scholar
- Giakoustidis A, Morrison D, Thillainayagam A, Stamp G, Mahadevan V. Ciliated foregut cyst of the gallbladder. A diagnostic challenge and management quandary. J Gastrointestinal Liver Disease. 2014;23(2):207–10.View ArticleGoogle Scholar
- Tunçyü Ö, Nart D, Yama B, Buyukcoban E. A ciliated foregut cyst in a gallbladder: the smallest reported. Jpn J Radiol. 2013;31:412–8.View ArticleGoogle Scholar
- Nam ES, Lee HI, Kim DH, Choi CS, Kim YB, Kim JS, et al. Ciliated foregut cyst of the gallbladder: A case report and review of literature. Pathol Int. 2000;50:427–30.View ArticlePubMedGoogle Scholar
- Kakitsubata Y, Kakitsubata S, Marutsuka K, Watanabe K. Epithelial cyst of the gallbladder demonstrated by ultrasonography: case report. Radiat Med. 1995;13(6):309–10.PubMedGoogle Scholar
- Baranger B, Poichotte A, Bernard P, Palazzo L, Deligny M, André JL. Kyste à revêtement cilié localisé à la voie biliaire principale. Ann Chir. 1998;52(1):98.PubMedGoogle Scholar
- Bulut AS, Karayalçin K. Ciliated foregut cyst of the gallbladder: report of a case and review of literature. Pathology Res International. 2010;193535:3.Google Scholar
- Wilson JM, Groesch R, George B, Turaga KK, Patel PJ, Saeian K, et al. Ciliated hepatic cyst leading to squamous cell carcinoma of the liver—a case report and review of literature. International J Surgery Case Reports. 2013;4:972–5.View ArticleGoogle Scholar
- Bishop KC, Perrino CM, Ruzinova MB, Brunt EM. Ciliated hepatic foregut cyst: a report of 6 cases and a review of the English literature. Diagn Pathol. 2015;10:81.PubMed CentralView ArticlePubMedGoogle Scholar