Introduction

 

Biliary cystadenomas are rare, benign but potentially malignant, multilocular, cystic neoplasms of the biliary ductal system. They usually arise in the liver (80-85%), accounting for less than 5% of cystic neoplasms of the liver.[1] Less than 100 reports of intrahepatic biliary cystadenomas are identified in medical literature.[2] In this case report, we present a patient with intrahepatic biliary cystadenoma (BCA) extending into the common bile duct (CBD), presenting as obstructive jaundice. Such occurrences are rare, and we review the imaging, diagnostic process and the surgical strategy.

 

Case Report

 

A 34-year-old lady was admitted with recurrent episodes of obstructive jaundice since 1 year. Liver function tests showed mild elevation of total bilirubin of 3.6 mg/dL; direct component: 2.1 mg/dL. Serum CA 19-9 was 35.9 U/mL. Echinococcal serology  was equivocal. MRCP showed short segment stricture in CBD,3.5 cm from the hilum with abrupt transition to normal caliber distally with moderate dilatation of intrahepatic ducts (Figure 1A). A well defined cystic lesion (4 x 4.5 cm) hypointense on T1 and hyperintense on T2 was seen in segment 4 with few thin internal septae, suspicious of hydatid cyst.

 

CECT revealed a suspicious intraluminal mid-CBD mass lesion, intrahepatic bile duct dilatation and segment 4 liver cyst (Figure 1B).

 

In view of CBD mass lesion associated with stricture, she was suspected to have a neoplastic mid CBD stricture along with segment 4 hydatid cyst of liver. Hence, she was planned for exploration for possible resection of the CBD lesion with adequate margins and clearance.

 

On exploration, upon transecting the CBD distally, a bile stained membranous mass protruded from the proximal dilated duct (Figure 2A & 2B). On further traction, it was found to be extending down from the segment 4 cyst. It was excised en toto along with the strictured segment of bile duct. Checkcholedochoscopy of the proximal biliary tree upto the second order division was normal. Upon probing the segment 4 cyst, it was seen communicating with the left main hepatic duct.

 

Frozen section of the excised CBD stricture was negative for malignancy and thought to be inflammatory due to internal rupture and leaking of hydatid fluid. Reconstruction was achieved by hepaticojejunostomy and the same roux-en-Y loop of jejunum was used to construct the adjacent cystojejunostomy, in view of the large biliary communication. She had an uneventful postoperative recovery.

 

Histology perplexed us as there was no evidence of hydatid cyst. Instead it showed cyst wall lined by a single layer of low cuboidal epithelium (Figure 3A) with the underlying stroma resembling ovarian stroma (Figure 3B) at places, reported as a  multilocular biliary cystadenoma. So, we concluded that this biliary cystadenoma, originating in the segment 4 duct  prolapsed into the bile duct and mimicked CBD mass and hydatid cyst. In view of the revised diagnosis, the patient has been advised completion surgery.

 

Discussion

 

BCA occurs most commonly in middle-aged females.[3,4] Episodic jaundice has only been reported in five patients in English literature.[5,6,7] Obstructive jaundice, although not always present, is the most frequent presenting symptom in patients with extrahepatic cystadenomas.[2] On the contrary, in intrahepatic cystadenomas, biliary obstruction is rarely the chief presenting complaint.[8]

 

There are only eight reported cases of intrahepatic BCA causing obstructive jaundice, either due to protruding polypoidal masses extending into a major duct or from intracystic haemorrhage or intraluminal mucin secretion by the tumour.[5,6,7] The most likely cause of episodic jaundice in this patient would have been the protruberant cyst extending into CBD, causing ductal dilatation as reported by Taketomi et al with reactive inflammatory biliary stricture, not described before in literature.[8]

 

BCA has two forms: the more common mucinous and the rare serous type. The former is further subdivided by the presence or absence of mesenchymal stroma. Mesenchymal stroma occurs exclusively in women.[3]

 

Pre-operative diagnosis can be difficult, but helps to strategize surgery. Biliary cystadenoma has been mistaken for hydatid cyst earlier.[10] On USG and CECT, BCAs appear as focal lesions with internal septae. Preoperative percutaneous biopsy has no additional value as it rarely produces a definitive diagnosis.[6] Intraoperative choledochoscopy is useful to assess  the ductal system, as exemplified by this case, helping to detect the origin of the intrahepatic component, which could be missed on preoperative imaging.[9] Frozen sections are not very useful due to the variability in histology of cystadenomas and their inability to rule out cystadenocarcinomas.[11] Careful histopathologic evaluation of the resected specimen, therefore, constitutes the only safe diagnostic modality.[6]

 

BCA has a high rate of recurrence and a potential for neoplastic transformation in approximately 10% of cases.[12] Cystadenoma without mesenchymal stroma is known to be more aggressive, especially in men.[3] In the past, treatment of BCA has included aspiration, marsupialization, internal drainage and partial excision. The main concerns of these methods are local recurrence, malignant transformation and misdiagnosis of cancer. The ideal treatment should be complete excision of the tumour which includes formal liver resection or wide local excision, as contemporary hepatic surgery has minimal morbidity and mortality.[13] Follow-up is conducted best by performing abdominal US or CT scan at 6-month intervals for the first postoperative year and then annually.[12]

 

Intrahepatic BCA can present with episodic surgical jaundice. Biliary cystadenoma should be suspected when radiologic imaging studies suggest a multilocular cystic hepatic lesion, especially in a woman.[14] Histopathological examination establishes definitive diagnosis. To the best of our knowledge, this is the first case of BCA with an associated biliary stricture.

 

References

1.     Marcial MA, Hauser SC, Cibas ES, Braver J. Intrahepatic biliary cystadenoma. Clinical, radiological, and pathological findings. Dig Dis Sci. 1986;31:884–8.

2.     Manouras A, Markogiannakis H, Lagoudianakis E, Katergiannakis V. Biliary cystadenoma with mesenchymal stroma: report of a case and review of the literature. World J Gastroenterol. 2006;12:6062–9.

3.     Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol. 1994;18:1078–91.

4.     Florman SS, Slakey DP. Giant biliary cystadenoma: case report and literature review. Am Surg. 2001;67:727–32.

5.     Beretta E, De Francis R, Staudacher C, Faravelli A, Primignani M, Vecchi M, et al. Biliary cystadenoma: an uncommon cause of recurrent cholestatic jaundice. Am J Gastroenterol. 1986;81:138–40.

6.     Erdogan D, Busch ORC, Rauws EAJ, van Delden OM, Gouma DJ, van Gulik TM: Obstructive jaundice due to hepatobiliary cystadenoma or cystadenocarcinoma. World J Gastroenterol. 2006;12:5735–8.

7.     Siriwardana PN, Pathirana A. Episodic biliary obstruction due to an intrahepatic biliary cystadenoma: a case report. J Med Case Reports. 2009;3:9032.

8.     Taketomi A, Tamada R, Takenaka K, Kawano R, Maeda T, Sugimachi K. A case of biliary cystadenoma with obstructive jaundice. Oncol Rep. 1998;5:833–5.

9.     Preetha M, Chung AYF, Lim-Tan SK, Lim DTH, Thug CH. Intrahepatic biliary cystadenoma presenting with obstructive jaundice. Asian J Surg. 2004;27:243–5.

10.   Raza MH, Rab AZ, Khan S, Ahmad R. Biliary cystadenoma mimicking hydatid cyst. Saudi J Gastroenterol. 2009;15:199–200.

11.   Vogt DP, Henderson JM, Chmielewski E. Cystadenoma and cystadenocarcinoma of the liver: a single center experience. J Am Coll Surg. 2005;200:727–33.

12.   Ariyathna K. Hepatic Cystadenomas. eMedicine Journal. 2001. Available at: http//www.emedicine.com/med/topic 492.htm.

13.   Lau WY, Chow CH, Leung ML. Total excision of mucinous biliary cystadenoma. Aust NZ J Surg. 1990;60:226–8.

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.