Ciliated Hepatic Foregut Cyst: 103 Cases in the World Literature

Chenwi Ambe,Steven C Cunningham,Nader Hanna,Luis Gonzalez-Cuyar,Saleem Farooqui

doi:10.4236/ojpathology.2012.23010

Chenwi Ambe, Steven C Cunningham + Show 3 more

Open Access

https://doi.org/10.4236/ojpathology.2012.23010

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Abstract

Ciliated hepatic foregut cysts (CHFC) are rare, typically benign, lesions whose incidence has been increasing recently. Despite this increase in incidence, they remain rare and several key characteristics remain poorly understood, including the range of presentation and the risk of malignant conversion. To better understand CHFC, an extensive review of the international literature was performed. Characteristics including size, location, contents, presenting symptoms, and risk of malignancy were analyzed. In addition, an illustrative case is presented to highlight a potential pitfall in diagnosis: Although the presentation is thought to be typically painless or vaguely painful, these lesions may also present with hemorrhage and sudden severe abdominal pain. Importantly, although malignant degeneration is uncommon, analysis revealed that malignancy is significantly associated with size, which was significantly larger (median 10 cm) in malignant CHFC compared with the typical benign CHFC (3 cm) (P < 0.01). Large or symptomatic cysts, or small asymptomatic cysts that are atypical, should be resected.

Highlights

Friedreich is credited with describing the first case of a ciliated hepatic foregut cyst (CHFC) in 1857 and positing their congenital origin [1]
CHFC Presenting with Severe, Sudden Pain of Intracystic Hemorrhage
CHFC is typically thought to be a benign lesion, and is typically discovered incidentally in the asymptomatic patient or in patients with vague chronic pain, the current analysis highlights the range both the wide range of presentation and the fact that malignancy may be present in the absence of arterial enhancement

Summary

Introduction

Friedreich is credited with describing the first case of a ciliated hepatic foregut cyst (CHFC) in 1857 and positing their congenital origin [1]. Wheeler and Edmonson were the first to use the term “ciliated hepatic foregut cyst” to describe the lesion [2] They identified specific characteristics which differentiate it from other hepatic cysts, such as the presence of four typical layers: a pseudostratified columnar epithelium layer with interspersed mucus cells, a subepithelial connective tissue layer, a smooth muscle layer and an outer fibrous capsule. This lesion is thought to be the result of evagination of the foregut during embryonogenesis. Imaging procedures demonstrated a cystic hepatic lesion in segment 4, prompting transfer to our hospital He had no history of recent trauma and reported no significant medical or surgical history. There was evidence of hemorrhage and extensive lymphoplasmacytic inflammation involving the cyst wall and the liver parenchyma, but no evidence of malignancy

Literature Review and Analysis

Discussion

Findings

Conclusion