Background: To report a rare case of adenocarcinoma arising from mucinous cystic neoplasm of liver.
Methods: This 71-year-old female presented in our institution with a palpable right upper quadrant abdominal mass. Computed tomography showed large multi-lobular complex cystic lesions of the right liver lobe with thick and irregular septa, raising the suspicion of a biliary cystadenoma.
Results: Extended right hepatectomy with cholecystectomy was done and pathology proved moderately differentiated adenocarcinoma arising from mucinous cystic neoplasm. The cyst was lined by columnar, cuboidal or flattened, mucus-secreting epithelium with scattered papillary projections and high-grade dysplasia. The ovarian-like stroma lies beneath the epithelium and estrogen receptor (ER) and inhibin α were also expressed. The immunostains of the tumor cells showed carcinoembryonic antigen (CEA)(focal +), ER(+), inhibin-α(+), CK7(−), S100P(−). She was discharge uneventfully and then attended regular follow-up without tumor recurrence for more than 2 years.
Conclusions: Mucinous cystic neoplasms of the liver (MCN-L) is a rare disease and its characteristics are still poorly understood. Radiologic features of various cystic liver lesions overlap, it is necessary to integrate imaging with clinical and laboratory findings to allow more definite diagnosis. Computerized tomography must be chosen for definitive diagnosis since the presence of septa, central septa, mural nodules and mucin-producing cystic neoplasms of the liver can be differentiated from solitary bile duct cysts with a high degree of accuracy. According to 2014 ACG clinical guideline imaging characteristics suggestive of BC (biliary cystadenoma) or BCA (biliary cystadenocarcinoma), such as internal septa, fenestrations, calcifications, or irregular walls, should lead to referral for complete surgical excision, and routine fluid aspiration is not needed because of limited sensitivity and the risk of malignant dissemination.
