Ciliated hepatic foregut cyst in a young child

Sunghoon Kima, Frances V. Whiteb, William McAlisterc,Ross Shepherdd, George Mychaliskaa,*

aDivision of Pediatric Surgery, Washington University School of Medicine, St. Louis, MO 63110, USAbDepartment of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110, USAcDepartment of Radiology, Washington University School of Medicine, St. Louis, MO 63110, USAdDivision of Pediatric Gastroenterology, Washington University School of Medicine, St. Louis, MO 63110, USA

Abstract Ciliated hepatic foregut cysts are a rare entity usually found in adults. We present a case of

a 3-year-old boy incidentally noted to have a radiographically complex liver cyst on computed

tomographic scan. Given the complex appearance, the cyst was excised. Pathology revealed a

ciliated hepatic foregut cyst. This is the second child and youngest patient affected with this lesion

reported in the literature. The etiology of the lesion and an argument for surgical removal in

pediatric patients are presented.

D 2005 Elsevier Inc. All rights reserved.

Ciliated hepatic foregut cyst (CHFC) is a rare, benign,

solitary, usually unilocular liver cyst distinguished by a

pseudostratified ciliated epithelial layer [1]. It is thought to

be derived from embryologic foregut, and Wheeler and

Edmonsdon [2] first proposed the name in 1984. They are

typically located in the left subcapsular hepatic lobe [3].

They are not associated with other congenital hepatic

lesions, have a slight male predominance, and usually are

diagnosed incidentally [3,5]. Sixty cases have been reported

in the literature in patients between 5 and 82 years of age,

with a mean of 52 years [4,5]. Because of the variable

mucinous content of cyst, computed tomographic (CT) scan

differentiation from simple cyst, parasitic cyst, cystade-

noma, and metastatic lesion can be difficult [6]. We describe

a 3-year-old male patient with CHFC whom we believe is

the youngest patient reported with this lesion.

1. Case report

A 3-year-old boy was referred for the evaluation of an

asymptomatic 2 � 1.5 cm cystic liver lesion found

incidentally on a CT scan (Fig. 1A). The CT scan was

initially performed to evaluate for occult spina bifida, which

was not found. Sonography confirmed a subcapsular cyst

with thin walls, internal septations, avascularity, and an

irregular shape in the right lobe of the liver (Fig. 1B). The

patient’s medical history was significant for persistent

eosinophilia since 2 years of age. Studies for Echinococcus

and E histolytica were negative.

Because of the uncertain etiology of the mass, surgical

exploration was undertaken. The lesion was localized

preoperatively by ultrasound, which facilitated a small

subcostal incision. The mass appeared multilocular and

0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved.

doi:10.1016/j.jpedsurg.2005.07.061

* Corresponding author. Division of Pediatric Surgery, St. Louis

Children’s Hospital, One Children’s Place, St. Louis, MO 63110, USA.

Tel.: +1 314 454 6022; fax: +1 314 454 2442.

E-mail address: kimsu@msnotes.wustl.edu (G. Mychaliska).

Index words:Ciliated hepatic foregut

cyst;

Child

Journal of Pediatric Surgery (2005) 40, E51–E53

www.elsevier.com/locate/jpedsurg

had a bluish hue. The lesion was completely excised. The

fluid within the cyst was viscous and dark. Microscopic

sections showed a 1.0-cm webbed cyst with a few smaller

cysts at its periphery (Fig. 2). The cyst was lined by

cuboidal and pseudostratified columnar epithelium includ-

ing ciliated and mucous cells, with underlying connective

tissue, focal smooth muscle bands, and fibrous capsule. The

patient’s postoperative course was uneventful.

2. Discussion

Ciliated hepatic foregut cyst is an uncommon cystic

lesion of the liver with only 60 cases reported in the

literature [4]. It is usually detected incidentally in adult

patients or at autopsies. A large number of patients with

CHFC have been reported in Japanese patients, but the

reason for this is unclear [5]. The only child with CHFC

reported in the literature was a 5-year-old girl described by

Carnicer et al [7] in 1996.

Radiographically, CHFCs are usually subcapsular or

peripheral and can be located in either lobe of the liver.

They are avascular, unilocular, or occasionally multi-

locular, round, or irregular cysts around 3 cm in diameter

without solid tissue in the walls. The lesions can be well

visualized by sonography, CT, or magnetic resonance

imaging. The mucoid or viscus material content in the

cyst can be confused with solid tissue on non–contrast-

enhanced CT. Magnetic resonance imaging can best

characterize the cyst.

The presence of ciliated columnar cells in a liver lesion is

pathognomonic for CHFC [1]. The cyst wall is composed of

cuboidal to pseudostratified epithelium containing ciliated

and mucous cells, subepithelial connective tissue, smooth

muscle, and a fibrous capsule. The cysts bear resemblance

to bronchogenic and esophageal cysts suggestive of their

common derivation from the embryonic foregut [2].

Bronchogenic cysts are characterized by ciliated pseudos-

tratified columnar epithelium, mucous glands, cartilage, and

smooth muscle bands. In contrast, esophageal cysts have

two distinct muscle layers, contain ciliated or squamous

epithelium, and lack cartilage and mucous glands. Cysts

without the defining characteristics of bronchogenic or

esophageal cysts are labeled as a foregut cyst. Because of

morphological and ultrastructural similarities to normal

bronchi, some have suggested that CHFC is an embryologic

foregut cyst, which has undergone bronchial differentiation

in the liver [8]. Ciliated cysts have been reported in other

foregut derived organs, such as in oral cavity, stomach,

pancreas, and gallbladder [8]. These cysts therefore

represent a spectrum of derivatives from embryologic

foregut and may be labeled as ciliated-specific organ-

foregut cysts.

Ciliated hepatic foregut cyst is considered to be an

asymptomatic, benign hepatic lesion. In the past 5 years,

however, 3 cases of squamous cell carcinoma arising from

CHFC have been described [9-11]. Similarly, squamous

cell carcinomas arising from bronchogenic and esophageal

cysts have been described in literature [12-16]. Although

there is controversy regarding the need for removal of

Fig. 1 A, Computed tomographic scan of the liver showing a

decreased attenuation mass consistent with a cyst in the right lobe.

B, Ultrasound image of liver shows an irregular sonolucent mass

with increased through transmission of a cyst.

Fig. 2 Histology of the cyst shows a lining of pseudostratified

columnar epithelium including ciliated and mucous cells, with

underlying connective tissue and smooth muscle (H&E, original

magnification �300).

S. Kim et al.E52

asymptomatic bronchogenic cyst, they are usually removed

because of the risk of malignancy. In light of the common

origin of CHFC and bronchogenic cyst, it is not surprising

that squamous cell carcinomas have been found to arise

from CHFC. Given the potential for malignant transfor-

mation in CHFC, we recommend removal of asymptomatic

CHFC in pediatric patients as well as in adults. Given their

small size and subcapsular location, they can be removed

with minimal morbidity.

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