Abstract

Biliary cystadenoma is a very rare cystic neoplasm of the liver that usually occurs almost exclusively in middle-aged women. These are slowly progressive tumours and cannot be safely differentiated from cystadenocarcinomas on preoperative imaging and hence should always be considered for resection. Historically these cystic tumors have been treated by a variety of techniques including aspiration, fenestration, internal drainage, partial resection resulting in high rates of recurrence. In addition biliary cystadenoma is a premalignant lesion and only surgical excision can differentiate it from its malignant counterpart, biliary cystadenocarcinoma.

|?Fig. 1: Triphasic computed tomography showing biliary cystadenoma with mutiloculations, septa, and mural nodule (arrow) and extension up to the pelvis

|?Fig. 2: Intraoperative photograph of the cyst and enucleation specimen

|?Fig. 3: (a and b) Sections showing cuboidal lining of cyst with subjacent cellular compact ovarian-like mesenchymal stroma, beneath which there is a fibrous layer (H and E). Immunohistochemistry showing nuclear positivity for (c) estrogen receptor and (d) progesterone receptor and cytoplasmic positivity for (e) smooth muscle actin in the ovarian-like stroma. On immunohistochemistry, (f) carcinoembryonic antigen is seen staining the luminal border of lining cells

Discussion

BCAs constitute?<5% of cystic space-occupying lesions of the liver. It occurs generally in middle-aged women presenting with abdominal pain and/or discomfort, distension, and sometimes a palpable mass.[1] [5] [11] The almost exclusive occurrence in females and associated increase in size during pregnancy and following oral contraceptives suggest hormonal dependency.[1] [2] [12]

BCAs are usually multiloculated, septated cystic tumors, and are of two types as follows: with[1] [6] ovarian-like and without ovarian-like stroma. Wheeler and Edmondson initially defined BCAs in 1958 as multilocular lesions with ovarian-like stroma, but subsequently, BCAs without ovarian stroma were reported. Cystadenomas with ovarian stroma are considered premalignant with a good prognosis, while those without ovarian stroma have a higher risk of transformation to malignancy and have a poorer prognosis.[6] [7] The majority of BCAs do not communicate with the bile ducts, but rarely it may be present.[2] Differential diagnosis of cystadenomas includes simple liver cysts, parasitic cysts (particularly hydatid cyst), liver abscess, polycystic liver disease, biliary cystadenocarcinoma, and cystic metastases.[7] [8] [11] Diagnosis of BCAs is often possible on imaging in the form of ultrasonography, CECT, and MRI abdomen. On all these imaging modalities, the presence of multiloculated cyst, septations, internal echoes, and mural nodules with papillary projections[7] [13] is typical. CECT, in addition, delineates the anatomic relation to the surrounding structures, particularly major vessels.[11] [13] In spite of all the advanced imaging modalities and the aforementioned radiological features, the preoperative radiological diagnostic accuracy may be as low as 30%, leading to misdiagnosis, and therefore, a high index of suspicion is needed.[14] A preoperative core needle biopsy or fine-needle aspiration cytology to detect malignancy is not recommended, especially in operable lesions due to nonaccuracy and the risk of needle seeding and dissemination.[15] [16] Elevated carcinoembryonic antigen and CA 19-9 in the serum or the cystic fluid may aid in diagnosis and follow-up of patients.[17] A normal level, however, does not exclude a BCA; hence, these procedures are not commonly done.

The important issues while making a diagnosis of BCA are the incomplete excision of the cyst, misdiagnosed as a simple cyst or a hydatid cyst, resulting in recurrence, and the difficulty in differentiating BCAs from biliary cystadenocarcinomas,[1] [7] [10] preoperatively or intraoperatively. Hence, the recommendation is to completely resect any suspected BCA.[1] [10] Since it closely resembles other cystic Space occupying lesion (SOLs) of liver, any suspicion intraoperatively warrants a resection. Intraoperative frozen section of the cyst is a useful aid in such situations as it can differentiate a BCA from other cystic lesions of liver but not biliary cystadenocarcinoma. Historically, BCAs have been treated with various procedures such as marsupialization, internal Roux-en-Y drainage, aspiration, or partial resection. However, all these procedures have been associated with high rates of recurrence.[5] [7] [12] [18] [19] Hence, complete resection is the recommended treatment with negligible recurrence.[6] Pinson?et al.[20] have reported cyst enucleation without late recurrence and mortality. This procedure is a valid alternative where resection is difficult or is likely to be associated with higher morbidity.[10]

In conclusion, the diagnosis of BCA should be considered in any multilocular cystic lesion of the liver, especially recurrent cysts, particularly in a middle-aged woman. The recommended treatment of choice for any suspected BCA is resection as it is extremely difficult to differentiate it from cystadenocarcinoma preoperatively. Enucleation is another feasible option and is indicated where resection is difficult due to the location of the tumor and proximity to hepatic hilum and major vessels.

|?Fig. 1: Triphasic computed tomography showing biliary cystadenoma with mutiloculations, septa, and mural nodule (arrow) and extension up to the pelvis

|?Fig. 2: Intraoperative photograph of the cyst and enucleation specimen

|?Fig. 3: (a and b) Sections showing cuboidal lining of cyst with subjacent cellular compact ovarian-like mesenchymal stroma, beneath which there is a fibrous layer (H and E). Immunohistochemistry showing nuclear positivity for (c) estrogen receptor and (d) progesterone receptor and cytoplasmic positivity for (e) smooth muscle actin in the ovarian-like stroma. On immunohistochemistry, (f) carcinoembryonic antigen is seen staining the luminal border of lining cells

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