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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 253-255
Intrahepatic biliary cystadenoma with ciliary metaplasia: Report of a rare morphological variant

1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Surgery, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication19-Jun-2017


Histological diagnosis of biliary cystadenoma is based on the type of epithelial cells lining the cyst and radiological features. The cyst-lining cells are not usually ciliated. We herein report a very rare example of an intrahepatic biliary cystadenoma with ciliated epithelial lining, which had taken us away from this diagnosis toward an intrahepatic foregut duplication cyst. Radiologically, also the lesion was deceptive, and a possibility of hydatid cyst was considered. However, immunohistochemical workup finally led us to this diagnosis. This report would document this rare morphological variant, which may pose diagnostic difficulty.

Keywords: Biliary cystadenoma, ciliated lining epithelium, intrahepatic cyst, magnetic resonance imaging

How to cite this article:
Khanna G, Sharma P, Madhusudhan K S, Barwad A, Ranjan P, Mishra B, Das P. Intrahepatic biliary cystadenoma with ciliary metaplasia: Report of a rare morphological variant. Indian J Pathol Microbiol 2017;60:253-5

How to cite this URL:
Khanna G, Sharma P, Madhusudhan K S, Barwad A, Ranjan P, Mishra B, Das P. Intrahepatic biliary cystadenoma with ciliary metaplasia: Report of a rare morphological variant. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 Aug 16];60:253-5. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/253/208394

   Introduction Top

Biliary cystadenoma is a rare benign cystic neoplasm of liver, constituting <5% of all nonparasitic hepatic cysts.[1] It is commonly seen in middle-aged females, with complaints of vague abdominal pain. Although radiological investigations play an important role, a tissue diagnosis is essential for confirmation, and to differentiate it from other hepatic cysts such as abscess, hydatid cyst, posttraumatic cyst, polycystic liver disease, and malignant cystic lesions.[2]

Here, we present a case of intrahepatic biliary cystadenoma (IHBCA) which on radiology mimicked a hydatid cyst and also caused a histological diagnostic dilemma due to the presence of unusual morphological features.

   Case Report Top

This 45-year-old female presented with a history of intermittent pain in the upper outer quadrant since last 5 months. Ultrasonography revealed a cystic lesion within the right lobe of the liver involving segments five and six. Routine blood investigations including liver function tests and serology for hydatid disease were normal. Previous records also disclosed history of a similar lesion in the subdiaphragmatic region and a borderline cystadenofibroma of left ovary. Both of these lesions were operated 8 years back at another hospital; however, operative detail could not be traced. Magnetic resonance imaging (MRI) scan performed revealed a multiloculated cystic lesion involving the segment seven and eight of liver, with a provisional diagnosis of hydatid cyst [Figure 1]a,[Figure 1]b,[Figure 1]c. Subsequently, operative excision was planned, and peroperatively multiloculated cysts were identified in the subcapsular region of segment seven and eight of liver. These cysts were not communicating with the biliary tree, and no cysts were identified in association with extrahepatic biliary tree or gallbladder. Macroscopically, the cysts had a smooth external surface and were filled with clear serous fluid. The inner surface was smooth, and no solid areas or papillary projections could be identified [Figure 1]d. Histopathological examination showed fibrocollagenous wall lined by cuboidal to columnar epithelial lining. At places, ovarian stroma was identified within the wall. However, the most interesting fact was that the cyst lining epithelium showed ciliated columnar lining, leading to an obvious provisional diagnosis of a ciliated foregut cyst [Figure 1]e,[Figure 1]f,[Figure 1]g. However, lack of smooth muscle in the cyst wall and subsequent immunohistochemical expression of CK 19, gave clues to the biliary differentiation of the cyst-lining epithelium [Figure 1]h. Stain for carcinoembryonic antigen (CEA) was negative in the lining epithelium, which is a marker of an usual ciliated hepatic foregut cyst. Till date, 4 months following surgery, the patient is asymptomatic.
Figure 1: Axial T1-weighted (a), axial T2-weighted (b), and coronal true-FISP (c) magnetic resonance images showing well-defined lobulated cystic lesion (appearing hypointense on T1-weighted and hyperintense on T2-weighted) in segments 7 and 8 of liver (arrows) with multiple internal septations. The gross photograph of the excised lesion confirming septations (d). Photomicrographs showing fibrocollagenous cyst wall, lined by low cuboidal to columnar epithelial cells with luminal ciliary projections (arrows) (e-g - H and E, e - ×40; f and g - ×200). The epithelium was strongly positive for CK19 (h – [CK19] ×200)

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   Discussion Top

Biliary cystadenoma is a rare benign cystic lesion and accounts for <5% of the cystic neoplasms of liver.[1],[3] Among the biliary cystadenomas, intrahepatic lesions are more common and constitute approximately 85%, with right lobe being the most frequent site (55%), followed by left lobe (29%) and rarely these lesions can be bilobar (16%). The extrahepatic lesions constitute remaining 15%.[4] Although this lesion can present at any age, middle-aged females (mean age 45 years) are most commonly affected, suggesting the possibility of hormonal pathogenesis.[1],[2],[5]

IHBCA is commonly asymptomatic and is detected incidentally. Clinically, they can present with vague abdominal discomfort, fullness, nausea, pain in upper abdomen, and rarely with obstructive biliary symptoms such as jaundice or cholangitis.[5]

Although the origin of IHBCA is unclear, they possibly arise from embryonal hepatobiliary stem cells or congenital aberrant bile duct.[6],[7]

Biochemical and serological findings are not diagnostic of IHBCA, but elevated serum levels of CEA and carbohydrate antigen 19-9 (CA 19-9) have been reported in some recent studies. CA 19-9 and CEA levels have also been studied in intracystic fluids with mixed results.[6],[7]

The preoperative assessment and exclusion of the other cystic lesions are important, as incomplete excision of IHBCA is associated with recurrence and malignant transformation.[8],[9] Radiology plays an important role in the diagnosis of all intrahepatic cystic lesions. Certain differential diagnoses which need to be excluded are hydatid cyst, simple cysts, liver abscesses, intraductal papillary mucinous tumor (IPMT), and biliary cystadenocarcinomas. Various clinical and radiological features can help in differentiating these lesions from IHBCA, for example, positive serology with peripheral blood eosinophilia is seen in hydatid cyst, communication with the intrahepatic biliary tract may favor the diagnosis of IPMT, and presence of a mural nodule, irregular enhancement of wall, or papillary areas may suggest malignancy.[10]

Hypoechoic or anechoic cystic lesion with internal septations is the common findings seen in IHBCA on ultrasonography. Hyperechoic shadows are suggestive of focal wall fibrosis, intracystic hemorrhage, or papillary areas.[10] Computed tomography (CT) scan also reveal well-demarcated multiloculated cystic lesion filled with mucin or blood with internal septations. In our particular case, the lesion radiological findings were elusive, and the radiological suggestion of a hydatid cyst was suggested on CT scan, as it cannot be distinguished from other cystic lesions without using contrast. MRI scan shows hypointense signal intensity on T1-weighted images and hyperintense on T2-weighted images.[10]

Tissue diagnosis of IHBCA is mandatory as the malignant counterpart cannot be ruled out completely on radiology.[9] Grossly, IHBCAs are large multiloculated cysts with smooth external and internal surface and contain clear serous or mucinous fluid. Rarely, the fluid can be hemorrhagic or purulent, if infected. Microscopically, the cyst wall is lined by a flat cuboidal to columnar nonciliated epithelium resting on the basement membrane and supported by hyalinized stroma closing of fibroblastic mesenchymal cells, ovary-like stroma, etc., seen in 85% of these cases.[1],[2],[3] Intraoperative frozen sections and fine-needle aspirations have been attempted in recent studies showed, however, false negative results and lack of reliability of these techniques.[2] In the index case, however, the lining epithelium showed clear ciliary projections, without any identifiable muscle bundles in the cyst wall. Immunohistochemical stains also helped us to reach to a final diagnosis of IHBCA with ciliary metaplasia of lining epithelium. The index case was further complicated by the previous history of the presence of ovarian cystadenofibromas where ciliary structures can be noted. Intrahepatic foregut duplication cyst also should be a diagnostic consideration.

   Conclusions Top

Biliary cystadenomas are rare cystic neoplasms, when intrahepatic, can cause diagnostic difficulty due to nonclassical radiological features and unknown morphological variations. Histological examination is mandatory, and if histological findings are found to be not classical, further immunohistochemical workup and correlation with biochemical tumor markers should be done. Ciliary metaplasia in an IHBCA is extremely rare, however, can be found.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Tsepelaki A, Kirkilesis I, Katsiva V, Triantafillidis JK, Vagianos C. Biliary Cystadenoma of the liver: Case report and systematic review of the literature. Ann Gastroenterol 2009;22:278-83.  Back to cited text no. 1
Al-Qahtani HH. Diagnostic uncertainty of hepatobiliary cystadenoma: Report of 11 cases and review of the literature.J Taibah Univ Med Sci 2016;11:19-25.  Back to cited text no. 2
Vogt DP, Henderson JM, Chmielewski E. Cystadenoma and cystadenocarcinoma of the liver: A single center experience. J Am Coll Surg 2005;200:727-33.  Back to cited text no. 3
Williamson JM, Rees JR, Pope I, Strickland A. Hepatobiliary cystadenomas. Ann R Coll Surg Engl 2013;95:507-10.  Back to cited text no. 4
Kuberan K, Chadrasekar G. Biliary cystadenoma of liver. Stanley Med J 2015;2:37-42.  Back to cited text no. 5
6. Dixon E, Sutherland FR, Mitchell P, McKinnon G, Nayak V. Cystadenomas of the liver: A spectrum of disease. Can J Surg 2001;44:371-6.  Back to cited text no. 6
Soochan D, Keough V, Wanless I, Molinari M. Intra and extra-hepatic cystadenoma of the biliary duct. Review of literature and radiological and pathological characteristics of a very rare case. BMJ Case Rep 2012;2012. pii: Bcr0120125497.  Back to cited text no. 7
Chen YW, Li CH, Liu Z, Dong JH, Zhang WZ, Jiang K. Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver. Genet Mol Res 2014;13:6383-90.  Back to cited text no. 8
Arnaoutaki DJ, Kim Y, Pulitano C, Zaydfudim V, Squires MH, Kooby D, et al. Management of biliary cystic tumors: A multi-institutional analysis of a rare liver tumor. Ann Surg 2015;261(2):361-7.  Back to cited text no. 9
Frahm C, Zimmermann A, Heller M, Brossmann J. Uncommon presentation of a giant biliary cystadenoma: Correlation between MRI and pathologic findings. J Magn Reson Imaging 2001;14:649-52.  Back to cited text no. 10

Correspondence Address:
Prasenjit Das
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.208394

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