Biliary cystadenoma: short- and long-term outcome after radical hepatic resection

Abstract

The intrahepatic biliary cystadenoma is a rare benign tumor of the liver, originating from an intrahepatic bile duct: it becomes symptomatic only when it causes obstruction of the bile duct itself. Regardless of the various diagnostic modalities available, it is difficult to distinguish preoperatively the cystadenoma both from a simple liver cyst, and from a cystic carcinoma of the bile duct. An incomplete surgical removal of the cyst often results in a higher risk of size increase and recurrence, even considering that the lesion may degenerate into a cystadenocarcinoma. Between January 2004 and May 2011, 1,173 liver resections were carried out at the Hepatobiliary Surgery Unit of San Raffaele Hospital: 12 of these were performed for cystadenoma. Forty-six patients underwent laparoscopic liver cysts deroofing: definitive histological examination in six of these patients revealed instead the diagnosis of cystadenoma. In 50% of cases, the diagnosis of cystadenoma was therefore acquired as a result of an incidental finding. The patients were all female, median age 45 years. The liver resection included six cases of left hepatectomy, three left lobectomies, and three of the right hepatectomy. The operations were performed by laparotomy, with the exception of two left lobectomies completed laparoscopically. In all cases, the postoperative course was without major complications. The resection was radical in all cases and the median hospital stay was 5 days. At a median follow-up of 16 months (range 7-30), all patients are alive and disease free. Biliary cystadenomas can easily be misunderstood and interpreted as simple hepatic cysts. Radical surgical resection is necessary and provides good short- and long-term outcomes.

Cystic Neoplasms of the Liver: Biliary Cystadenoma and Cystadenocarcinoma

“…(43, 52, 59) The combination of imaging characteristics and serologic analysis along with culture of cyst fluid can aid in making an accurate diagnosis. (43, 61)…”

Arrangements of Hepatobiliary Cystadenoma Complicated With Congenital Choledochal Cyst

“…It is difficult to make an accurate diagnosis of biliary cystadenomas before surgery, and till date no publication has established presenting symptoms that differentiate biliary cystadenomas from other benign or malignant hepatic cystic diseases such as biliary cystadenocarcinoma, hepatic and hydatid cyst, Caroli disease, undifferentiated sarcoma, intraductal papillary mucinous tumor, and hepatocellular carcinoma. 11 In this case, the patient had been misdiagnosed as having a simple cyst and did not receive a timely surgery. The typical CT features of cystadenoma are usually a well-defined mass with low-density and internal septa.…”

“…Patients may present with abdominal pain and distention, dyspepsia, anorexia, nausea, and vomiting; however, patients may be asymptomatic at presentation. 11 Jaundice is either due to an extrinsic compression of the bile duct, biliary obstruction by an intraluminal tumoral mass, or accretion of mucus secretion from a communicating biliary cystadenoma. 12 Patient presented with fever and mild jaundice due to the improper discharge of jelly-like bile leading to the increased pressure of the bile duct and bacterial infections.…”

Giant biliary cystadenoma

“…Abnormal embryonic development resulting in ectopic foregut or gonadal epithelium sequestered in the liver has been proposed (3,4). Presenting symptoms include abdominal pain, per os intolerance, abdominal distention, nausea, vomiting, and jaundice, yet patients may be asymptomatic at presentation (3-6). As in other reports, our patient presented with abdominal pain and decreased per os intake.…”

“…Cystotomy and internal drainage of IHBCA has been reported but carries risks of potential malignant degeneration of the residue, recurrence, and peritoneal seeding in the case of intrahepatic biliary cystadenocarcinoma (IHBCAC). The presence of mesenchymal stroma within an IHBCA increases the risk of malignant transformation and supports resection rather than conservative treatment (6,10). The presence of ovarian stroma within our patient’s IHBCA further justified resection due to increased malignant potential.…”