CASE 13885 Published on 05.10.2016

Biliary cystadenoma with bile duct communication demonstrated on contrast-enhanced hepatobiliary phase MRI sequence

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

José Pablo León¹, Cristina La Parra¹, Ulises Guajardo²

(1) Department of Radiology,
Hospital Universitario de La Ribera,
Alzira, Valencia, Spain.
(2) School of Medicine,
Universidad de los Andes,
Santiago, Chile.

Patient

17 years, female

Categories

Area of Interest Abdomen, Liver ; Imaging Technique MR, MR-Functional imaging, Ultrasound, CT, CT-High Resolution

Procedure Diagnostic procedure ; Special Focus Neoplasia ;

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Clinical History

We present the case of a healthy 17-year-old woman who presented with a history of intermittent upper right abdominal pain for several months. Physical examination was unremarkable and laboratory findings were within normal range.

Imaging Findings

Abdominal ultrasound and CT (Fig. 1) showed a large and well-defined multiloculated cystic lesion in the IV hepatic segment with enhancing septations without any solid pole or calcifications in the wall. A mild intrahepatic biliary ducts dilatation was also detected. MRI study with hepatobiliary-specific contrast agent and MRI cholangiography was performed (Fig. 2, 3). On T1 WI sequence the lesion was mildly hyperintense. On T2 WI and contrast-enhanced venous phase sequence the lesion showed similar findings as CT, confirming a large hepatic multiloculated cystic lesion with enhancing septations without solid pole. On contrast-enhanced hepatobiliary phase sequence contrast accumulated in the lesion was detected, a finding consistent with communication to the right principal biliary duct. These findings were compatible with biliary cystadenoma. Complete surgical excision was performed and bile duct communication was confirmed. The pathological study confirmed the diagnosis of biliary cystadenoma.

Discussion

Background
Biliary cystadenomas are rare neoplasms of the biliary system [1]. Approximately 85% of cases appear within the intrahepatic bile duct; the others occur either within the extrahepatic biliary tree or gallbladder [2].

Clinical Perspective
It is usually slowly growing in size and its clinical presentation is rather variable and unspecific. Biliary cystadenomas occur predominantly in middle-aged women [3] and may potentially transform into cystadenocarcinomas, therefore complete surgical excision is the treatment of choice [4].

Imaging Perspective
At CT and MR imaging, a typical biliary cystadenoma or cystadenocarcinoma appears as a large, solitary, multilocular cystic lesion with well-circumscribed smooth margins and internal septa [3]. The distinction between biliary cystadenoma and cystadenocarcinomas remains difficult [5]. The presence of septa and nodules suggest cystadenocarcinoma [2]. Ultrasound and CT are usually the initial imaging tests for localization and characterization of the tumour [6]. MRI could be a more specific technique for diagnosis [7]. The contrast-enhanced hepatobiliary phase MRI sequence allows visualization of the bile duct communication facilitating the diagnosis, as noted in our case. Moreover, Zen Y et al. [8] examined the pathological features of biliary cystic tumours with communication to the bile duct. Their results suggested that biliary cystic tumour with bile duct communication might be a cystic variant of Intraductal Papillary Neoplasm of the Bile Duct (IPN-B) rather than a true biliary cystic neoplasm.
The differential diagnosis of biliary cystadenoma is extensive. Liver abscess and hydatid cysts are the two disorders most likely to be confused with. Distinguishing infected collections from cystic malignancies relies on clinical and laboratory analysis. MRI usually shows a thicker wall in liver abscesses with round or oval daughter cysts in hydatid cysts. Concerning the simple cysts, MRI often shows the lack of the septations and nodules. Intrahepatic haematoma may also appear cystic; however, former liver trauma or known hepatocellular adenoma may allow to reach the diagnosis. Rarely, cystic metastatic or cystic primary hepatocellular carcinoma will simulate biliary cystoadenoma in patients with a known metastatic disease or with liver cirrhosis, respectively [5].

Outcome
Biliary cystadenomas may potentially transform into cystadenocarcinomas, therefore complete surgical excision is the treatment of choice [4].

Take Home Message, Teaching points
The differential diagnosis of biliary cystoadenoma is extensive. The use of liver-specific contrast media that demonstrate a communication between the lesion and the biliary tree may aid in assessing the origin of a cystic tumour from the biliary ducts facilitating the diagnosis [9].

Differential Diagnosis List

Biliary cystadenoma

Biliary cystadenocarcinoma

Hydatid cyst

Hepatic abscess

Haemorrhagic and simple hepatic cyst

Metastases

Final Diagnosis

Biliary cystadenoma

References

[1] Mortelé KJ, Ros PR. (2001) Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics 21(4):895-910. (PMID: 11452064)

[2] Buetow PC, Buck JL, Pantongrag-Brown L, et al. (1995) Biliary cystadenoma and cystadenocarcinoma: clinical- imaging-pathologic correlations with emphasis on the importance of ovarian stroma. Radiology 196(3):805–810. (PMID: 7644647)

[3] Qian LJ, Zhu J, Zhuang ZG, Xia Q, Liu Q, Xu JR. (2013) Spectrum of multilocular cystic hepatic lesions: CT and MR imaging findings with pathologic correlation. Radiographics 33(5):1419-1433. (PMID: 24025933)

[4] Läuffer JM, Baer HU, Maurer CA, Stoupis C, Zimmerman A, Büchler MW. (1998) Biliary cystadenocarcinoma of the liver: the need for complete resection. Eur J Cancer 34(12):1845-1851. (PMID: 10023304)

[5] Lewin M, Mourra N, Honigman I, Fléjou JF, Parc R, Arrivé L, Tubiana JM. (2006) Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur Radiol 16(2):407-413 (PMID: 15983777)

[6] Korobkin M, Stephens DH, Lee JK, Stanley RJ, Fishman EK, Francis IR et al. (1989) Biliary cystadenoma and cystadenocarcinoma: CT and sonographics findings. Am J Roentgenol 153: 507–511. (PMID: 2669463)

[7] Williams DM, Vitellas KM, Sheafor D. (2001) Biliary cystadenocarcinoma: seven year follow-up and the role of MRI and MRCP. Magn Reson Imaging 19(9):1203-1208. (PMID: 11755730)

[8] Zen Y, Fujii T, Itatsu K, Nakamura K, Konishi F, Masuda S et al. (2006) Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct. Mod Pathol 19(9):1243-1254. (PMID: 16741522)

[9] Marrone G1, Maggiore G, Carollo V, Sonzogni A, Luca A (2011) Biliary cystadenoma with bile duct communication depicted on liver-specific contrast agent-enhanced MRI in a child. Pediatr Radiol 41(1):121-4 (PMID: 20585769)

Case information

URL:	https://www.eurorad.org/case/13885
DOI:	10.1594/EURORAD/CASE.13885
ISSN:	1563-4086

License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

A: Abdominal ultrasound. B-C Axial and coronal CT venous phase.

The images show a large and well-defined multiloculated cystic lesion in the IV-hepatic-segment with enhancing septations without any solid pole or calcifications in the wall. It also shows mild intrahepatic biliary ducts dilatation.

Figure 2

MRI. A: T1WI B: T2WI C: Contrast-enhanced

On image A the lesion is mildly hyperintense. Images B and C show a hepatic multiloculated cystic lesion with enhancing septations without any solid pole and a mild intrahepatic biliary ducts dilatation.

Figure 3

MRI. A - B: Contrast-enhanced hepatobiliary phase sequences C: Cholangiography-sequence

On images A and B a contrast accumulation inside the lesion is detected, a finding that is consistent with communication to the biliary duct. The image C show a mild intrahepatic biliary ducts dilatation.