Background/aims: Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCACa) are rare cystic neoplasms that usually arise from the liver. We reviewed the clinicopathologic and radiologic findings of 13 cases of intrahepatic biliary cystic neoplasms.

Methods: Seven patients with BCA and 6 patients with BCACa which were pathologically proven within past 10 years were included in this retrospective study.

Results: BCA (7 of 7) was more common in female compared to BCACa (4 of 6). Mean age at diagnosis was 53.4 years (BCA) and 58.5 years (BCACa). Abdominal pain (54%) was the most common presenting symptom. Eleven patients (61.5%) exhibited normal liver function profiles and 5 patients (38%) showed elevated levels of serum CA19-9 levels (mean 894.2 U/mL, range: 78.7-2,080). Mean size of tumor was 11.7 cm (range: 5-15). Most frequent radiologic finding was a single cystic mass with septation. BCACa tended to have intracystic solid portion. The cut surface revealed a unilocular or multilocular cystic mass with mucinous contents. Complete surgical excision was done in 12 patients. After the complete resection, recurrence was observed in 1 case of BCACa.

Conclusions: The possibility of biliary cystic neoplasm should be suspected when an intrahepatic cystic lesion with multiseptation or solid portion is noted on imaging study. In addition, complete excision for definite diagnosis and treatment need to be performed.

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