Ciliated hepatic foregut cysts: A case series and literature review

Abstract

Background: Ciliated hepatic foregut cysts (CHFCs) are rare, uniloculate, cystic lesions of embryological origin and are of significance due to their malignant potential. They are the only ciliated cystic lesions known to occur in the liver, tend to be asymptomatic and are found more frequently in male patients. They are often incidental findings on imaging or during surgery, typically in segment IV of the liver. This study highlights two cases of CHFCs managed at a regional tertiary centre in 2018, and reviews the current literature on CHFCs. Material and Methods: A PubMed search for CHFCs was completed.Of the aforementioned two patients with CHFCs, relevant clinical information was obtained from electronic patient records. Results: The literature review yielded 81 papers. Although the exact aetiology is unknown, several reports hypothesize that CHFCs originate from detachment and migration of buds from the oesophageal and bronchial regions of the foregut that are entrapped by the liver during embryonic development. CHFCs are mostly found in segments IVA and IVB, and have unique histology; ciliated pseudostratified columnar epithelium. The mechanism of malignant transformation is unclear but rate of transformation is estimated at 6%. Case 1: A 47 year old fit and healthy male whose CHFC was identified incidentally on imaging, underwent laparoscopic segment IV hepatic resection with CHFC confirmed histologically. Case 2: A 64 year old male with haemangiopericytoma of the brain, who developed a liver metastasis; also haemangiopericytoma. His CHFC was an incidental finding wholly excised during an ALPPS procedure for resection of this metastasis. Conclusion: CHFCs are rare lesions. The malignant transformation risk is based on anecdotal case series data. The evidence for optimum management is lacking; and a patient-centred choice between long term follow-up or definitive laparoscopic resection must be made. Recommendation for surgery is based on level 3 evidence.