Mucinous cystic neoplasm of the liver (MCN-L): a rare presentation and review of the literature

Abstract

Mucinous cystic neoplasms of the liver (MCN-L, (previously referred to as cystadenomas or cystadenocarcinoma) are rare cystic tumors that occur within the liver parenchyma, or less frequently, in the extrahepatic bile ducts. They are reported to account for <5% of all liver cysts. The differential diagnosis of MCN-L includes intraductal papillary neoplasm of the bile duct (IPNB), intrahepatic cholangiocarcinoma with cystic change, echinococcal cyst, and a simple cyst. Invasive MCNs can only be differentiated from non-invasive MCNs by microscopic evaluation for the presence of ovarian-type stroma. Intraoperative biopsy and frozen section(s) are essential to differentiate MCNs from other cystic liver lesions. The treatment of choice is complete excision and can result in excellent survival with initial correct diagnosis. But its rare presentation and insufficient recognition frequently lead to an incorrect initial or delayed diagnosis or misdiagnosis.

Keywords: cystadenoma; differential diagnosis; liver; mucinous cystic neoplasms.

Figure 1

CECT abdomen: well-defined unilocular cyst 14 × 12 cm noted in left lobe of liver causing mass effect which is seen as rightward displacement and compression of gall bladder.

Figure 2

Histopathology findings: cyst lined by layer of bland, cuboidal to low columnar mucinous epithelium. The underlying stromas composed of fibrocollagenous tissue with collections of histiocytes along with densely packed spindle cells, histologically mimicking ovarian-type stroma.