Intrahepatic biliary mucinous cystic neoplasms: clinicoradiological characteristics and surgical results

Abstract

BackgroundIntrahepatic biliary mucinous cystic neoplasms are rare hepatic tumors and account for less than 5% of intrahepatic cystic lesions. Accurate preoperative diagnosis is difficult and the outcome differs among various treatment modalities.The aim of this study is to investigate the clinico-radiological characteristics of intrahepatic biliary mucinous cystic neoplasms and to establish eligible diagnostic and treatment suggestions.MethodsNineteen patients with intrahepatic biliary cystadenomas and two patients with biliary cystadenocarcinomas were retrospectively reviewed. Their clinico-radiological variables and survival outcome were analyzed.ResultsOf the 19 patients with biliary cystadenoma, 16 (84.2 %) were female. 11 (57.9 %) patients had symptoms before operation with the most common presenting symptom being abdominal pain. Among the patients with available data, serum and cystic fluid CA 19–9 levels were invariably elevated and the CA 19–9 level in the cystic fluid was significantly higher than that in the serum. Loculations (84.2 %) and septations (63.2 %) were the most common radiologic findings. For treatment, 11 (57.9 %) patients received radical resection by either enucleation or hepatic resection, while the remaining 8 (42.1 %) patients underwent only fenestration of liver cysts. Radical resection provided a significantly better clinical outcome than fenestration in terms of tumor recurrence (p = 0.018). The only two male patients with biliary cystadenocarcinoma received radical hepatic resection and achieved a disease-free survival of 16.5 months and 33 months, respectively.ConclusionIntrahepatic biliary mucinous cystic neoplasms are rare and preoperative diagnosis is difficult. Internal septations and loculations on radiologic examinations should raise some suspicion of this diagnosis. Complete tumor excision is the standard treatment that may provide patients with better long term results after the operation.

Simultaneous occurrence of hepatic hydatid cyst and mucinous cystadenoma of the liver in a middle-aged female patient: report of a rare case

“…The presence of a residual cyst 1 month after the initial surgery of hepatic cystectomy performed laparoscopically could indicate that the initial treatment may have been inadequate. In the absence of operative notes of the initial surgery, we can only conjecture that the cyst was not excised totally but may have undergone a fenestration and partial cystectomy leading to recurrence 15. A case was reported similar to ours, wherein an inadequately excised MCN-L in one institution underwent a formal hepatectomy following rapid recurrence in another institution.…”

“…Laparoscopic resection of hepatic cysts is the recommended treatment for benign hepatic cysts 2. But inadequate resection can lead to recurrence 15. The presence of a residual cyst 1 month after the initial surgery of hepatic cystectomy performed laparoscopically could indicate that the initial treatment may have been inadequate.…”

A giant and insidious subphrenic biloma formation due to gallbladder perforation mimicking biliary cystic tumor: A case report

“…Although rare, BCTs should be suspected for a cystic neoplasm of liver, particularly with septum-like structures and suspicious intracystic components, as in our case study (21). However, hematological analysis and tumor markers are non-specific for distinguishing BCTs from other hepatic cystic masses (21,22). Given the suspected BCT, image-guided needle aspiration cytology or biopsy is not implemented, due to high false negative results and the risk of peritoneal dissemination (23).…”

Long-term follow-up of intrahepatic biliary cystadenoma and cystadenocarcinoma following hepatectomy

“…BCA can become malignant, with an extremely high local relapse rate in cases of noncomplete or partial resection; thus, radical resection is the preferred treatment. Palliative surgical approaches, such as lesion puncture (to withdraw fluid), injection of hardening agents, internal drainage, open drainage, and partial resection, are contraindicated and should be avoided [3,5,81,14] . The BCA relapse rate is 48.6% after open drainage and partial resection but only 10% after an extensive hepatectomy [3] .…”