• Biliary cystadenocarcinoma
• Biliary cystadenoma
• Hepatic simple cyst
• Imaging modalities
• Mucinous cystic neoplasms

• Computed tomography
• Cystadenoma, Mucinous
• Cysts
• Liver
• Magnetic resonance imaging

• Bile duct
• Liver
• Mucinous cystic neoplasm
• Papillary neoplasm

• Humans
• Female
• Male
• Diagnosis, Differential
• Middle Aged
• Bile Duct Neoplasms/diagnostic imaging
• Bile Duct Neoplasms/classification
• Aged
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/classification
• Adult
• Retrospective Studies
• Sensitivity and Specificity
• Tomography, X-Ray Computed/methods

• Biliary mucinous cystic neoplasms
• High-grade intraepithelial neoplasms
• Low-grade intraepithelial neoplasms
• Overall survival
• Recurrence-free survival

• Humans
• Female
• Middle Aged
• Male
• Aged
• Adult
• Bile Duct Neoplasms/mortality
• Bile Duct Neoplasms/pathology
• Bile Duct Neoplasms/surgery
• Bile Duct Neoplasms/diagnosis
• Bile Duct Neoplasms/therapy
• Bile Duct Neoplasms/blood
• Retrospective Studies
• CA-125 Antigen/blood
• CA-19-9 Antigen/blood
• Biomarkers, Tumor/blood
• Biomarkers, Tumor/analysis
• Neoplasms, Cystic, Mucinous, and Serous/pathology
• Neoplasms, Cystic, Mucinous, and Serous/therapy
• Neoplasms, Cystic, Mucinous, and Serous/mortality
• Neoplasms, Cystic, Mucinous, and Serous/surgery
• Neoplasms, Cystic, Mucinous, and Serous/blood
• China/epidemiology
• Bile Ducts, Intrahepatic/pathology
• Bile Ducts, Intrahepatic/surgery
• Treatment Outcome

• Diagnosis
• Invasive carcinoma
• Liver
• Mucinous cystic neoplasms
• Surgical resection
• Treatment

• Humans
• Female
• Male
• Middle Aged
• Retrospective Studies
• Liver Neoplasms/diagnosis
• Liver Neoplasms/pathology
• Liver Neoplasms/therapy
• Liver Neoplasms/surgery
• Adult
• Aged
• Liver/pathology
• Liver/surgery
• Liver/metabolism
• Liver/diagnostic imaging
• Treatment Outcome

• Humans
• Focal Nodular Hyperplasia/diagnosis
• Focal Nodular Hyperplasia/pathology
• Liver Neoplasms/diagnosis
• Liver Neoplasms/pathology
• Liver Neoplasms/therapy
• Liver Neoplasms/diagnostic imaging
• Liver Diseases/diagnosis
• Liver Diseases/therapy
• Liver Diseases/diagnostic imaging
• Liver Diseases/pathology
• Hemangioma/diagnosis
• Hemangioma/therapy
• Hemangioma/pathology
• Hemangioma/diagnostic imaging
• Cysts/diagnosis
• Cysts/diagnostic imaging
• Cysts/pathology
• Adenoma, Liver Cell/diagnosis
• Adenoma, Liver Cell/pathology
• Adenoma, Liver Cell/therapy
• Adenoma, Liver Cell/diagnostic imaging
• Diagnosis, Differential
• Gastroenterology/standards
• Carcinoma, Hepatocellular/diagnosis
• Carcinoma, Hepatocellular/pathology
• Carcinoma, Hepatocellular/therapy
• Carcinoma, Hepatocellular/diagnostic imaging

• Biliary mucinous cystic neoplasm
• Cyst
• Hemangioma
• Liver

• Cyst
• Liver
• Pathology
• Radiology
• Surgery

• Humans
• Retrospective Studies
• Pancreatic Neoplasms/pathology
• Neoplasms, Cystic, Mucinous, and Serous
• Cysts
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/surgery

• MD/PhD scholarship Amsterdam University Medical Centers

• Biliary cystadenocarcinoma
• Clinicopathological criteria, Surgical outcomes
• Mucinous biliary cystic tumors, Biliary cystadenoma

• Humans
• Male
• Retrospective Studies
• Neoplasms, Cystic, Mucinous, and Serous
• Cholangiocarcinoma
• Bile Duct Neoplasms
• Bile Ducts, Intrahepatic
• Liver Neoplasms/surgery

• Mansoura University

• Algorithms
• Cystadenocarcinoma
• Cystadenoma
• Liver
• Magnetic resonance imaging
• Neoplasms, cystic, mucinous, and serous

• Humans
• Female
• Male
• Retrospective Studies
• Pancreatic Neoplasms/pathology
• Cysts/diagnostic imaging
• Liver Neoplasms/diagnostic imaging
• Neoplasms, Cystic, Mucinous, and Serous
• Algorithms

• Biliary cystadenocarcinoma
• Biliary cystadenoma
• Biliary mucinous cystic neoplasm
• Intraductal papillary neoplasm of the liver and bile ducts
• Mucinous cystic neoplasm of the liver and bile ducts
• Simple cyst

• Humans
• Neoplasms, Cystic, Mucinous, and Serous
• Gastrointestinal Neoplasms
• Magnetic Resonance Imaging
• Biomarkers, Tumor
• Cysts/diagnostic imaging
• Pancreatic Neoplasms/surgery

• hepatobiliary cystadenocarcinoma
• hepatobiliary cystadenoma
• mucinous cystic neoplasm

• biliary cystadenocarcinoma
• cystic tumor
• hepatic cyst
• hepatic tumor

• Animals
• Dogs
• Female
• Bile Duct Neoplasms/diagnostic imaging
• Bile Duct Neoplasms/surgery
• Bile Duct Neoplasms/veterinary
• Bile Ducts, Intrahepatic/pathology
• Cholangiocarcinoma/pathology
• Cholangiocarcinoma/veterinary
• Cystadenocarcinoma/diagnosis
• Cystadenocarcinoma/surgery
• Cystadenocarcinoma/veterinary
• Cystadenoma/diagnosis
• Cystadenoma/pathology
• Cystadenoma/surgery
• Cystadenoma/veterinary
• Dog Diseases/diagnostic imaging
• Dog Diseases/surgery

• Cysts/diagnosis
• Cysts/therapy
• Hamartoma
• Humans
• Liver Diseases/diagnosis
• Liver Diseases/therapy

Biliary cystadenoma is a rare cystic neoplasm of the liver. The clinical signs and symptoms are nonspecific, and treatment strategy is variable.

In this study, we presented a case of a 32-year-old female with multilocular biliary cystadenoma. The patient underwent partial removal of the hepatic cyst two times in two different hospitals for two years and that the histopathological results were biliary cystic adenoma but was successfully treated by radical resection after the second recurrence. The patient underwent a J-shaped laparotomy. The giant cystic mass measuring 20 cm × 15 cm was below the position of the right anterior segment. This lesion pushed the liver parenchyma to both sides and compressed the hepatic hilum, causing dilatation of the intrahepatic bile ducts. The patient underwent complete resection of cystic mass. During the dissection, a 0.5mm-diameter fistula of left hepatic duct with the cyst was found. It was sutured using absorbable polydioxanone (PDS 6.0) and the cystic duct tube (C tube) (6 Fr) was inserted via the cystic duct into the left hepatic duct due to drain the bile fluid.

A biliary cystadenoma (BCA) primary origin is occasionally rare. Although imaging modalities such as ultrasound, computed tomography and magnetic resonance imaging could be suggestive, however, the definitive diagnosis is depended on the histological examination. Despite of being a benign tumor, it has a high risk of recurrence after conservative treatment. The potential risk for malignant is also present. Therefore, complete resection of the tumors is the treatment of choice.

We herein present a report of a rare case with had a giant biliary cystadenoma (BCA) primary origin. This report aims to improve the understanding of the diagnosis and management of this uncommon disease.

•

A biliary cystadenoma (BCA) primary origin is occasionally rare, the clinical manifestations are variable and non-specific.

• Biliary cystadenocarcinoma
• Biliary cystadenoma
• Mucinous cystic neoplasm
• Radiographic imaging
• Simple liver cyst

• Humans
• Liver/pathology
• Magnetic Resonance Imaging/methods
• Mucocele
• Pancreatic Neoplasms/pathology
• Retrospective Studies
• Tomography, X-Ray Computed/methods

• P30 CA008748 NCI NIH HHS

• Biliary cystadenocarcinoma
• cryoablation
• microwave ablation
• sclerotherapy

• Abdominal Pain
• Aged
• Cystadenocarcinoma/diagnosis
• Cystadenocarcinoma/pathology
• Cystadenocarcinoma/surgery
• Cystadenoma/diagnosis
• Cystadenoma/pathology
• Cystadenoma/surgery
• Female
• Humans
• Polidocanol
• Quality of Life
• Sclerotherapy
• Ultrasonography

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary neoplasm of the bile duct (IPN-B) are two different types of mucin-producing bile duct tumour that may complicate the course of pregnancy. To the best of our knowledge, we describe herein the first case of MCN-L with spontaneous rupture during pregnancy necessitating complex surgical treatment.

A 24-year-old woman was initially admitted to another hospital in October 2018 with signs of jaundice (serum bilirubin level 12 mg/dL) and upper abdominal pain radiating to the left shoulder. Initial magnetic resonance imaging (MRI) of the abdominal cavity revealed a multilocular cystic tumour of the liver hilum (37 mm × 40 mm in diameter) located between segments 3 and 4 of the left liver lobe. Six weeks later (December 2018), the patient was found to be 12 wk pregnant and was referred to our institution for further diagnostics and treatment. At admission, a soft, palpable, and tender mass in the left upper abdomen was found. It was determined via MRI (with no intravenous contrast in view of the first-trimester pregnancy) to be a large collection of fluid (19 cm × 17 cm × 10 cm) located close to the liver hilum and below the left liver lobe. The patient did not undergo any diagnostic or therapeutic procedures nor did they have any abdominal trauma in the preceding weeks. The fluid collection proved to be of biliary origin following percutaneous drainage. Therefore, we concluded this was a spontaneous rupture of an MCN-L with the formation of a biloma. The MRI study also revealed the previously found cystic tumour of the liver hilum communicating with the left hepatic duct, which, together with left hepatic duct dilatation, suggested the diagnosis of IPN-B. The follow-up MRI with intravenous gadolinium contrast performed in the second trimester of pregnancy (week 14) showed, in turn, some features of MCN-L, including enhancement of the internal septations within the cystic liver mass. A precise preoperative differential diagnosis between IPN-B and MCN-L was therefore not possible. The patient was submitted to surgery in the second trimester of pregnancy (week 18). Surgery included a cholecystectomy, left hepatectomy, and concomitant resection of the extrahepatic bile ducts followed by anastomosis of the right hepatic duct with the Roux limb of the jejunum. The post-operative period was uneventful and the patient was discharged 8 days after surgery. The histopathological examination of the resected specimen revealed a final diagnosis of MCN-L with low-grade dysplasia and epithelium surrounded by ovarian-type stromal tissue. The patient delivered a healthy baby girl and both remain well at present, after 2 years of follow-up since surgery.

The differential diagnosis and management of MCN-L and IPN-B may be very challenging, particularly in the setting of pregnancy. When indications for surgery are obvious, the final diagnosis is based on histopathological examination, with ovarian-type stroma being pathognomonic for MCN-L. We believe that the growth of this subepithelial stroma secondary to the high levels of sex hormones produced during pregnancy might have been the main causative factor leading to the tumour rupture with the formation of a biloma in our patient.

• Biliary tract neoplasms
• Liver neoplasms
• Hepatectomy
• Anastomosis, Roux-en-Y
• Pregnancy
• Case report

• aspiration
• diagnosis
• fenestration
• management
• simple hepatic cyst

Apatinib is a new oral tyrosine kinase inhibitor that targets vascular endothelial growth factor receptor-2. It has been proven effective in treating multiple solid tumors. Herein, we report the case of a 67-year-old Chinese patient who was diagnosed with recurrent and malignant transformation of intrahepatic biliary cystadenoma. After multidisciplinary team discussion, the team considered that the remaining liver volume was insufficient for surgical resection. The patient refused chemotherapy and radiotherapy and was willing to take apatinib. Initially, the patient experienced severe tongue ulcers and difficulty eating. The dose of apatinib was then adjusted to 250 mg/day. To date, he has been taking apatinib for 48 months. Regular re-examination showed that the tumor had significantly decreased in size. On January 16, 2021, a CT scan revealed a tumor diameter of 4.5 cm. In our case, the patient achieved partial response and progression-free survival(PFS) of 48.0 months. During treatment, the patient’s appetite and mental state were expected. The treatment did not induce hypertension, fatigue, hand-foot syndrome, or liver and kidney damage. Apatinib may be an option for the treatment of advanced intrahepatic biliary cystadenocarcinoma. Its toxicity is controllable and tolerable. The exact curative effect still needs to be evaluated in more cases.

• apatinib
• intrahepatic bile duct cystic tumor
• intrahepatic biliary cystadenocarcinoma
• intrahepatic biliary cystadenoma
• targeted therapy

Hepatobiliary mucinous cystic neoplasms (H-MCNs) are relatively rare cystic neoplasms in the liver. The differential diagnosis of H-MCNs remains big challenging, and the management and prognosis between the hepatic simple cyst (HSC) and H-MCNs are quite different. This study aimed to present our experience in the management of H-MCNs and provide a preoperative H-MCNs risk prediction nomogram to differentiating H-MCNs from liver cystic lesions.

29 patients diagnosed with H-MCNs and 75 patients diagnosed with HSC between June 2011 and June 2019 at Zhejiang University School of medicine, Sir Run-Run Shaw Hospital were reviewed in this study. We analyzed the demographic and clinicopathological variables.

US, CT, and MRI could accurately diagnose only 3.4%, 46.1%, and 57.1% of H-MCNs, respectively. After univariate analysis and multivariate logistic regression analysis, the variables significantly associated with H-MCNs were enhancement after contrast (p = 0.009), tumour located in the left lobe (p = 0.02) and biliary ductal dilation (p = 0.027). An H-MCNs risk predictive nomogram was constructed, which showed excellent discrimination (areas under the receiver operating characteristic curve were 0.940) and consistent calibration between the predicted probability and actual probability.

Among patients with H-MCNs, the location of the tumour, enhancement in CT scan, and biliary duct dilation are significantly independent risk factors. The appropriate treatment of H-MCNs is radical resection. Using our Nomogram could facilitate screening and identification of patients with liver cystic lesions.

• Cystadenoma
• Diagnosis
• Liver
• Surgical procedures

• Biliary cystadenocarcinoma
• Biliary cystadenoma
• Biliary mucinous cystic neoplasm
• Mucinous cystic neoplasm
• Simple cyst

• Cysts/diagnosis
• Cysts/therapy
• Humans
• Liver Diseases/diagnosis
• Liver Diseases/therapy
• Quality of Life
• Sclerotherapy/adverse effects
• Treatment Outcome

• hepatic
• mucinous cystic neoplasm
• septations
• simple biliary cyst

• CA19.9
• CA72.4
• Hepatic cystadenocarcinoma
• Hepatic cystadenoma
• Simple hepatic cyst

• Cistoadenocarcinoma
• Cistoadenoma
• Cystadenocarcinoma
• Cystadenoma
• Cystic liver neoplasm
• Intraductal papillary biliary neoplasms
• Mucinous neoplasm
• Neoplasia mucinosa quística
• Neoplasia papilar intraductal biliar
• Neoplasia quística hepática

• Liver surgery
• cystic tumour
• intraductal papillary neoplasm of the bile duct
• mucinous cystic neoplasm

Cancers of the biliary tract include intra- and extrahepatic cholangiocarcinomas and gallbladder cancer. Biliary tract cancers are diseases with unfavorable prognoses. In recent years, several lesions have been described as precursors that precede biliary cancers. They include flat and microscopic lesions known as biliary intraepithelial neoplasia, macroscopic and tumor-forming intraductal papillary biliary neoplasia, intraductal tubular neoplasia, and mucinous cystic neoplasm of the bile duct. These conditions are rarely diagnosed, while their natural history and progression to cancer have yet to be adequately characterized. This review examines the epidemiology, pathology, molecular biology, diagnosis, and therapy of these various precursors. Further research is required if we are to better understand this evolving field and improve the prevention and early detection of bile duct cancer.

• Duodenum
• Gastric outlet obstruction
• Hepatic cyst
• Liver

Biliary mucinous cystic neoplasms are rare cystic lesions of the liver which carry pre-malignant potential. Given the scarcity of reports in the literature, they pose a considerable challenge to clinical management, particularly with regards to accurate pre-operative diagnosis.

We present the case of a 37-year-old Tunisian woman who presented with subacute right upper quadrant pain and a large multi-loculated cystic lesion, most consistent with a hydatid cyst. She underwent an open right hepatectomy, and pathology surprisingly revealed a biliary mucinous cystadenoma. Herein, we review the current literature on biliary mucinous cystic neoplasms, with a particular emphasis on diagnostic investigations, key radiological features and optimal treatment modalities.

Biliary mucinous cystic neoplasms require a high index of suspicion and should be managed with complete surgical resection, as conservative techniques are associated with high recurrence rates. Considering the potential for malignant transformation, periodical surveillance imaging is recommended in the post-operative period.

• Biliary cystadenoma
• Biliary mucinous cystic neoplasm
• Hydatid disease
• Investigation
• Management

• Biliary cystadenocarcinoma
• Biliary cystadenoma
• Invasive biliary mucinous neoplasm
• Liver cysts
• Liver resection

Mucinous cystic neoplasm of the liver with extrahepatic growth is a rare benign epithelial neoplasm of the biliary system that presents with a mass effect or is incidentally found on imaging. The tumor affects mostly the common hepatic duct, which is difficult to diagnose preoperatively by radiology, endoscopy, or cystic fluid analysis. Endoscopic ultrasound is a noninvasive tool for the evaluation of features of a cystic lesion and the extent of disease. Optimal treatment is complete tumor resection.

A 27-year-old Thai woman was referred to our hospital for investigation and treatment of clinical symptoms of obstructive jaundice and ascending cholangitis, as well as an unknown cause of obstruction. Multiple investigations were performed, including endoscopic retrograde cholangiography and magnetic resonance imaging. Endoscopic ultrasound showed a multiloculated cystic lesion with internal septations without communication to the bile duct, which helped to support a diagnosis of mucinous cystic neoplasm. Eventually, the pathological diagnosis made was mucinous cystic neoplasm of the bile duct. A follow-up clinical examination with imaging at 6 months revealed that the patient was asymptomatic and without recurrence.

We report a rare case of a patient with a large mucinous cystic neoplasm of the liver with extrahepatic growth causing biliary obstruction, which was diagnosed on the basis of endoscopic ultrasound features. Following definitive diagnosis, treatment with complete surgical resection using a multidisciplinary approach was successful.

• Ascending cholangitis
• Biliary cystadenoma
• Mucinous cystic neoplasm of the liver with extrahepatic growth

• congenital hepatic cyst
• intracystic hemorrhage
• liver resection

• Adult
• Cysts/complications
• Cysts/congenital
• Cysts/surgery
• Diagnosis, Differential
• Female
• Hemorrhage/diagnosis
• Hemorrhage/etiology
• Hemorrhage/surgery
• Hepatectomy/methods
• Humans
• Liver Diseases/complications
• Liver Diseases/congenital
• Liver Diseases/surgery
• Magnetic Resonance Imaging
• Middle Aged
• Tomography, X-Ray Computed

• Biliary cystadenoma
• Cyst recurrence
• Fenestration
• Hepatic cyst

Surgical resection is generally considered the main curative treatment for intrahepatic biliary cystadenocarcinoma (IBCA) or suspected IBCAs, but controversy exists regarding the prognosis for IBCAs. This study aimed to describe the clinicopathological characteristics of IBCA and identify prognostic factors that may influence the survival of patients treated with surgical procedures.

Thirty-four patients with histologically confirmed IBCA treated between January 2000 and June 2014 were included. The clinical characteristics of patients with IBCA were compared with those of 41 patients with intrahepatic biliary cystadenoma (IBC); factors that significant difference were analyzed for prognosis analysis of IBCA using multivariate/univariate Cox proportional hazards regression models. Survival curves were constructed using the Kaplan–Meier method and compared using the log-rank test.

IBCAs had a strong female predominance, and the most common presenting symptoms were abdominal pain or discomfort. Compared with IBCs, IBCAs occurred in older patients, in more male patients, and were associated statistically significant abnormal increase in alanine aminotransferase (P = 0.01) and total bilirubin (P = 0.04). Mural nodules were more frequently seen with IBCAs and may associate with malignancy. It was difficult to differentiate between IBC and IBCA based on laboratory examination and imaging findings. Although complete resection is recommended, enucleation with negative margins also achieved good outcomes. Median overall patient survival was 76.2 months; survival at 1, 3, and 5 years was 88.0%, 68.7%, and 45.8%, respectively. Radical resection and noninvasive tumor type were independent prognostic factors for overall survival.

It remains difficult to distinguish between cystadenomas and cystadenocarcinomas based on laboratory examination and image findings. Complete resection is recommended for curative treatment, and patients should be closely followed postoperatively, particularly those with invasive tumors.

• cystadenoma
• cystadenocarcinoma
• biliary cystic neoplasms

• Adult
• Aged
• Aged, 80 and over
• Bile Duct Neoplasms/diagnosis
• Bile Duct Neoplasms/diagnostic imaging
• Bile Duct Neoplasms/mortality
• Bile Duct Neoplasms/surgery
• Bile Ducts, Intrahepatic/diagnostic imaging
• Bile Ducts, Intrahepatic/surgery
• Cystadenocarcinoma/diagnosis
• Cystadenocarcinoma/diagnostic imaging
• Cystadenocarcinoma/mortality
• Cystadenocarcinoma/surgery
• Cystadenoma/diagnosis
• Cystadenoma/diagnostic imaging
• Cystadenoma/mortality
• Cystadenoma/surgery
• Female
• Follow-Up Studies
• Hepatectomy/methods
• Humans
• Male
• Middle Aged
• Preoperative Period
• Radiography
• Retrospective Studies
• Survival Analysis
• Treatment Outcome

Benign liver tumors are common. They do not spread to other areas of the body, and they usually do not pose a serious health risk. In fact, in most cases, benign liver tumors are not diagnosed because patients are asymptomatic. When they are detected, it’s usually because the person has had medical imaging tests, such as an ultrasound (US), computed tomography (CT) scan, or magnetic resonance imaging (MRI), for another condition.

A search of the literature was made using cancer literature and the PubMed, Scopus, and Web of Science (WOS) database for the following keywords: “hepatic benign tumors”, “hepatic cystic tumors”, “polycystic liver disease”, “liver macroregenerative nodules”, “hepatic mesenchymal hamartoma”, “hepatic angiomyolipoma”, “biliary cystadenoma”, and “nodular regenerative hyperplasia”.

Hepatocellular carcinoma (HCC) is one of the most common malignant tumors in some areas of the world; there is an increasing incidence worldwide. Approximately 750,000 new cases are reported per year. More than 75 % of cases occur in the Asia-Pacific region, largely in association with chronic hepatitis B virus (HBV) infection. The incidence of HCC is increasing in the USA and Europe because of the increased incidence of hepatitis C virus (HCV) infection. Unlike the liver HCC, benign tumors are less frequent. However, they represent a chapter always more interesting of liver disease. In fact, a careful differential diagnosis with the forms of malignant tumor is often required in such a way so as to direct the patient to the correct therapy. In conclusion, many of these tumors present with typical features in various imaging studies. On occasions, biopsies are required, and/or surgical removal is needed. In the majority of cases of benign hepatic tumors, no treatment is indicated. The main indication for treatment is the presence of significant clinical symptoms or suspicion of malignancy or fear of malignant transformation.