Intrahepatic biliary cystadenoma mimicking hydatid cyst of liver: a clinicopathologic study of six cases

Abstract

Background: Intrahepatic biliary cystadenomas are rare hepatic neoplasms, which are usually cystic. These tumors are often misdiagnosed as simple liver cysts and hydatid cysts clinically and radiologically owing to nonspecific clinical and radiologic features. These tumors require complete resection, as recurrence and malignant transformation can occur following incomplete excision. It is essential that these tumors be diagnosed accurately so that they can be adequately excised.

Methods: Clinical and radiological features of six cases of biliary cystadenoma are described.

Results: All of these cases were resected with the clinical and/or radiological impression of simple liver cysts and/or hydatid cysts. Out of the six patients, five were female and one was male. Ages of the patients ranged from 28 to 60 years (mean 45 years). The patients presented with nonspecific symptoms. Internal septations were seen on preoperative imaging (when available). On gross examination, all tumors were cystic; their sizes varied from 5.5 to 14 cm, mean size was 9.0 cm. On histopathologic examination, cystic spaces were lined by cuboidal to columnar mucin-secreting epithelium with underlying ovarian-type stroma. In one case, ovarian-type stroma was not seen. Recurrence was seen in three cases at 1 to 5 years of follow up.

Conclusions: Owing to their malignant potential and high recurrence rate following incomplete resection, an aggressive surgical approach is recommended. Prognosis is excellent after complete resection.

Keywords: Biliary cystadenoma; Cyst; Hydatid cyst; Internal septations; Liver.

Fig. 1

Axial and coronal sections from contrast-enhanced computed tomography of the abdomen. a There is a well-circumscribed, intrahepatic cystic lesion having thin walls (bold arrow). There are no internal septations, mural nodules, or abnormal wall enhancement, favoring diagnosis of cystadenoma. b There is significant associated mass effect resulting in compression over duodenum (thin arrow)

Fig. 2

Axial and sagittal sections from computed tomography of the abdomen. a There is a large, well-circumscribed, multiloculated, intrahepatic cystic lesion. Fine internal septations without enhancing component are appreciated (thin arrow). b There is associated mass effect resulting in splaying of branches of portal vein and intrahepatic biliary dilatation (thick arrow)

Fig. 3

a The cyst wall is lined by columnar epithelium with cytoplasmic mucin and subepithelial ovarian-type stroma (hematoxylin and eosin, 400 × magnification). b Strong immunoreactivity of anti-smooth muscle actin in ovarian-type stroma

Fig. 4

Axial and coronal sections from contrast-enhanced computed tomography of the abdomen. a There is a well-circumscribed, multiloculated, intrahepatic cystic lesion (bold arrow). The few areas of high attenuation probably represent hemorrhage within the lesion (thick arrow). b There is significant mass effect with moderate intrahepatic biliary dilatation (thin arrow)

Fig. 5

Histology of biliary cystadenoma of the single male patient of the series. a The cyst lining is columnar with cytoplasmic mucin. b Ulceration of the lining with fibrinopurulent exudate covering. No ovarian-type stroma is seen