Ciliated hepatic foregut cyst: a report of 6 cases and a review of the English literature

Abstract

Background: Ciliated hepatic foregut cyst (CHFC) is a rare cystic lesion most commonly identified in segment 4 of the liver that arises from the embryonic foregut. The classic histologic pattern is comprised of 4 distinct layers (inner ciliated epithelial lining, smooth muscle, loose connective tissue, fibrous capsule). Although rare, cases of metaplastic and malignant epithelial lining have been described in CHFC.

Methods: We report 6 additional cases of CHFC, one of which had gastric metaplasia of the cyst lining, and review all reported cases of CHFC in the English literature. We describe the clinicopathologic analysis of 6 cases, with selective immunohistochemical analysis on 1 case with gastric metaplasia.

Results: Cases occurred in 4 women and 2 men (average age 55 years, range 42 to 67 years). Cysts ranged in size from 0.7 to 17 cm (average 7.2 cm) and were grossly tan-pink to white with blood-filled contents. The majority were located in segment 4 of the liver, however 2 were located in the porta hepatis. Tumor serologies (CA19-9 and/or CEA) were performed in 3 cases; 1 case demonstrated elevated CA19-9, and 2 cases had laboratory values within normal limits. All cases showed the classic histologic findings, however one case additionally had extensive gastric metaplasia.

Conclusions: In conclusion, CHFC is a rare diagnostic entity that should be considered in the differential diagnosis for cystic hepatic lesions, particularly those located in segment 4 of the liver. Metaplasia and squamous carcinoma can occur, therefore complete surgical excision is the recommended treatment.

Fig. 1

CHFC, classic cases a Case 2, low power view highlights classic cyst architecture. b Case 3, CHFC is surrounded by a fibrous capsule and well-demarcated from the surrounding hepatic parenchyma. c Case 3, innermost layer may be completely or incompletely lined by cilia. d Case 4, rare goblet cells (black circle) mimicking respiratory tract mucosa may be seen. e Case 6, ciliated, pseudostratified epithelium. f Case 6, high power view shows pseudostratified, ciliated epithelium with abundant goblet cells (all images hematoxylin-eosin; original magnifications X40 [A], X100 [B], X200 [E], X400 [C, D, F])

Fig. 2

CHFC with gastric metaplasia a Case 4, CHFC with gastric-type mucus-secreting glands in the submucosa. b Case 4, CHFC with branching mucous glands. c Case 4, surface foveolar-type epithelium. d Case 4, classic ciliated, pseudostratified, epithelial cyst lining (all images hematoxylin-eosin; original magnifications X100 [A], X200 [B], X400 [C, D])