Prolapse into the bile duct and expansive growth is characteristic behavior of mucinous cystic neoplasm of the liver: report of two cases and review of the literature

Abstract

Mucinous cystic neoplasm of the liver (MCN-L) is a very rare tumor whose detailed behavior is still unknown. We describe two cases of MCN-L that exhibited extremely interesting growth patterns, and discuss the characteristics of MCN-Ls. Both cases exhibited MCN-L that originated from the left hepatic lobe (Segment 4) and then prolapsed into the left hepatic duct and common bile duct, resulting in obstructive jaundice due to expansive growth. Endoscopic retrograde cholangiopancreatographies showed the characteristic oval-shaped filling defects in the bile ducts. Endoscopic ultrasound and intraductal ultrasound were useful for differentiating the tumors from stones, since multiple septal formations were observed inside the tumors. A literature search revealed that, over the past 10 years, 15 cases of MCN-L (biliary cystadenomas with ovarian-like stroma) that showed expansive growth in the bile duct had been reported. Prolapse into the bile duct and expansive growth appear to be characteristic behavior of MCN-L. In the future, additional data on more cases needs to be collected to further elucidate MCN-L pathophysiology.

Mucinous cystic neoplasm of the liver with biliary communication: an exception to the current classification

“…Possible mechanisms for jaundice are intraductal extension, intracystic haemorrhage or mucin secretion. Very few case reports have described cystadenoma prolapsing into bile duct 11. In these two cases, it is due to intraductal extension of tumour which is a rare feature of MCN.…”

Mucinous Cystadenoma: A Rare Hepatic Tumor in a Child

“…In cases that present with severe jaundice, prolapse into the bile ducts may be noted. Because of the rapid onset of symptoms, masses with this growth pattern seem to be diagnosed at earlier ages ( 12 ). Results of liver tests, CA19-9 and CEA are typically normal ( 3 , 10 ).…”

Mucinous cystic neoplasms of the liver and pancreas: relationship between KRAS driver mutations and disease progression

“…Mucinous cystic neoplasms (MCNs) of the pancreatobiliary system are defined as cystic neoplasms consisting of the lining columnar epithelium and subepithelial cellular ‘ovarian‐like’ stroma . MCNs of the pancreas (MCNs‐P) are more common than MCNs of the liver (MCNs‐L) . Both tumours share clinicopathological features, including a female predominance, histological appearance, and lack of communication with the duct system .…”

“…[1][2][3][4][5][6] MCNs of the pancreas (MCNs-P) are more common than MCNs of the liver (MCNs-L). [7][8][9][10][11] Both tumours share clinicopathological features, including a female predominance, histological appearance, and lack of communication with the duct system. [2][3][4][5][6][7][8][9][10][11][12] MCNs are some of the main precursors of invasive adenocarcinoma in either organ, with the other two being intraductal papillary neoplasms and microscopic intraepithelial neoplasia.…”

“…[7][8][9][10][11] Both tumours share clinicopathological features, including a female predominance, histological appearance, and lack of communication with the duct system. [2][3][4][5][6][7][8][9][10][11][12] MCNs are some of the main precursors of invasive adenocarcinoma in either organ, with the other two being intraductal papillary neoplasms and microscopic intraepithelial neoplasia. [1][2][3][4][5][6][7][8][9][10][11][12] They also show multistep progression from low-grade dysplasia to invasive carcinoma, and are currently graded into four categories: non-invasive MCNs with low-grade, intermediate-grade or high-grade dysplasia, and those with an invasive carcinoma.…”