Introduction

 

Biliary cystadenoma (BCA) and cystadenocarcinoma are rare cystic tumours arising from the biliary epithelium. Their location is usually intrahepatic and they are characterized by large multiloculated macroscopic lesions. They rarely communicate with the biliary ductal system. Clinical features of biliary cystadenoma, due to its rarity, have been sparsely investigated, mostly from both surgical and radiological aspects. There have been fewer than 150 case reports of biliary cystadenoma and fewer than 50 cases of biliary cystadenocarcinoma in the literature so far.

 

Case Report

 

A 55-year-old woman presented in surgical clinic with complaints of lump in abdomen and abdominal pain for last one year. Physical examination showed a firm and cystic mass of 30 cm × 20 cm in the right upper quadrant reaching up to the pelvis. The patient was anemic but not icteric at the time of presentation with no peripheral lymphadenopathy.

 

Ultrasound abdomen showed a mixed echo of 20 cm × 12 cm × 15 cm in the left lobe of the liver, and blood flow was found inside the separations. CT scan abdomen showed a multiloculated cystic mass of 24 cm × 15 cm in the left lobe of liver, with low uneven density and clear edge, with a possibility of hepatic hydatid cyst. The organs around the tumor were pressed aside (Figure 1). Laboratory examination showed normal CA125, CA19.9, a-fetoprotein (AFP), carcinoembryonic antigen (CEA) and liver function tests. Keeping in mind the possibility of a giant hepatic hydatid cyst patient was subjected to exploratory laparotomy. During the operation, a large multiloculated cystic lesion was observed in the left lobe of liver, with a size of 23 cm × 20 cm × 18 cm. Its surface was dark red with abundant blood supply, and the capsule was intact (Figure 2). The lesion was arising from the left hepatic duct (Figure 3).

 

No other lesions in the liver or any evidence of ascites were noted. Complete excision of the mass with repair of left hepatic duct was done. On histological examination, the inner layer of the cyst was lined with columnar to cuboidal epithelial cells taking a papillary structure, with lack of nuclear atypia or mitoses, suggestive of benign cystadenoma (Figure 4). Post operatively she recovered well and was discharged on the seventh post-operative day. She is doing well during consecutive follow up.

 

Discussion

 

Biliary cystadenoma is a rare cystic neoplasm, accounting for fewer than 5% of all neoplasms of the bile duct. They arise from the intrahepatic or, less frequently, the extrahepatic bile duct.[1] The exact etiology of biliary cystadenoma is unknown.  The tumor is thought to result from the development of ectopic rests of primitive foregut sequestered within the liver or it is due to the obstruction of the congenitally aberrant bile duct.[2] Biliary cystadenoma is a true proliferative epithelial tumor that is histologically known to be insidiously progressive and takes years to enlarge. Most of these tumours are macroscopically multi-loculated and large, occasionally exceeding 10 cm in largest diameter. They may contain mucinous fluid produced  by mucin-secreting columnar epithelium.[3,4] Clinical manifestations of the tumor are not specific and can be variable.

 

They predominantly occur in middle-aged females and frequently cause abdominal pain and or a palpable mass.[5,6] These manifestations may be seen earlier if secondary infection, rupture of the tumor, or intracapsular bleeding occur. When the tumor invades the porta hepatis or compresses the extrahepatic bile duct, obstructive jaundice may be the presenting feature.[7,8] Ascites has been recognized as a sign of cystadenoma after tumor compression to the vena cava or hepatic veins. Images of the biliary cystadenoma demonstrate a large, fluid-filled, well-defined mass with multiple septa or papillary projections. The existence of solid nodular masses or coarse calcifications in the shell and septa suggests malignancy. Angiography demonstrates an avascular mass with small clusters of abnormal vessels in the periphery, although it is not of much diagnostic value. Endoscopic retrograde cholangiopancreatography (ERCP) may be useful to investigate if there is communication between the tumor and bile ducts.

 

Ultrasonography (US) and CT are feasible for the diagnosis of mucinous biliary cystadenoma.[9,10,11,12] The characteristic CT  findings of these tumours are low density, well-defined masseswith internal septa and mural nodules. The internal septa and wall usually show enhancement with intravenous contrast medium. US will usually show a cystic mass with multiple echogenic septa and or papillary projections along the wall or septa.[12,13] US is considered superior to CT in the demonstration of internal morphology of the mass. Tumor markers are not significant to distinguish between cystadenoma and cystadenocarcinoma. In the past, treatment of BCA has included aspiration, marsupialization, internal drainage and partial excision, probably due to poor preoperative imaging and the lack of understanding of BCA’s premalignant potential.

 

Radical excision of the tumor is the first choice of treatment. As mucinous cystadenoma is considered to be a premalignant lesion, operative removal is essentially important.[14] Biliary cystadenoma is almost confined to a certain lobe or section of the liver. If the liver tissue around the tumor is nearly normal and the liver function is good, surgery should be done. Mucinous biliary cystadenoma can be radically resected along with the tissue with a 2 cm margin from it.

 

In our patient the symptoms were non-specific, CT scan showed internal septations so the patient was operated with the probable diagnosis of hepatic hydatid cyst. Radical resection of the cyst was done because of its compression effect on the adjacent organs.

 

References

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10.   Choi BI, Lim JH, Han MC, Lee DH, Kim SH, Kim YI, et al. Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. Radiology. 1989;171:57–61.

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12.   Korobkin M, Stephens DH, Lee JK, Stanley RJ, Fishman EK, Francis IR, et al. Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. AJR Am J Roentgenol. 1989;153:507–11.

13.   Federle MP, Filly RA, Moss AA. Cystic hepatic neoplasms: complementary roles of CT and sonography. AJR Am J Roentgenol. 1981;136:345–8.

14.   Thomas KT, Welch D, Trueblood A, Sulur P, Wise P, Gorden DL, et al. Effective treatment of biliary cystadenoma. Ann Surg. 2005;241:769–73; discussion 73–5.