Introduction:
Biliary or hepatic cystadenoma (BCA) is a benign, premalignant neoplasm that often originates in the intrahepatic bile ducts, lined by secreting cuboidal or columnar epithelium. About 10 to 20 percent of biliary cystadenoma may arise from the gallbladder and extrahepatic bile ducts, constituting about five percent of all hepatic cystic lesions. The malignant form of biliary cystadenoma is biliary cystadenocarcinoma (BCAC) which arises from the premalignant lesion. The etiology of BCA is not exactly known, and it was suggested that it might arise from ectopic embryonic tissue or the embryonic rest of the primitive foregut.
What Is the Pathophysiology of the Biliary Cystadenoma?
Biliary cystadenoma appears as globular in shape, and its size ranges from 0.59 to 13.78 inches The external surface of the mass is smooth and contains multilocular cysts filled with mucinous fluid that are separated by thick walls. BCA appears as a single or multilocular cystic lesion in which the locules are lined by columnar epithelium with inflammation, hemorrhage, and severe dysplasia. Sometimes the dysplastic epithelium inside the bile duct causes obstruction of the biliary duct.
What Are the Symptoms of Biliary Cystadenoma?
Most patients with biliary cystadenoma remain asymptomatic. However, some of the patients experience the following symptoms:
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Abdominal pain.
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Abdominal distension.
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Nausea (urge to vomit).
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Vomiting.
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Compression of the duodenum, stomach, biliary tree, and hepatic veins.
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In extrahepatic BCT, obstructive jaundice and cholangitis (swelling of the bile duct) may be seen.
What Are the Imaging Techniques Used in the Diagnosis of Hepatic Cystadenoma?
1. Computed Tomography (CT):
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In CT, hepatic cystadenomas appear hypodense, and multilocular mass measures as large as 11.81 inches. The wall of the mass is well defined and appears enhanced with the administration of a contrast agent.
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Cystadenomas have thin septal calcifications and demonstrate fluid attenuation such as blood, bile, and mucin.
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The calcifications inside the mass appear thick and coarse. Sometimes there is a papillary projection into the lumen.
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Biliary dilatation is rarely seen in extrahepatic cystadenoma or cystadenocarcinoma.
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A CT scan can detect the presence of air inside the hepatic mass.
2. Magnetic Resonance Imaging (MRI):
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The fluid containing cystadenomas appears as low signal intensity homogeneous mass on T1-weighted images and high-intensity homogeneous mass on T2-weighted images. Based on the protein content of the fluid, the signal intensity may vary in T1 and T2 weighted images. Therefore, it is difficult to distinguish biliary cystadenomas from cystadenocarcinoma in MRI.
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The low-intensity signal inside the high-intensity cyst in T2 weighted images indicates septations.
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The combination of diffusion-weighted MRI with conventional MRI helps in the quantitative and qualitative assessment of hepatic lesions, including cysts and malignancy.
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MRI can distinguish abscesses from benign biliary cystadenomas with the presence of pericystic edema.
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The presence of air inside the hepatic abscess indicates air forming organism, and it appears as a signal void in MRI.
3. Ultrasonography:
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With the administration of ultrasound contrast materials, most of the lesion appears in a honeycomb pattern with cystic wall and septa. During the arterial phase, the honeycomb septa appear hyper-enhanced, most commonly in biliary cystadenomas. During portal venous and late phase, the honeycomb septa appear hypo enhanced in biliary cystadenocarcinoma.
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The biliary projections and mural nodules may project into the cystic lumen. The wall or septal classification appears as an acoustic shadow in ultrasonography.
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The cystic contents such as mucin, blood, or protein appear anechoic to hypoechoic.
What Are the Clinical Investigations Used in the Diagnosis of Biliary Cystadenoma?
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Elevated serum CA 19-9 and carcinoembryonic antigen (CEA) level.
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Elevated cystic fluid CA 19-9 and CEA levels help distinguish biliary cystadenoma and cystadenocarcinoma from various cystic lesions.
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Percutaneous fine-needle aspiration of cystic fluid is used to estimate the bilirubin level. Increased bilirubin level in cystic fluid indicates the communication of biliary cystadenoma with the biliary tract.
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It is difficult to differentiate biliary cystadenoma from cystadenocarcinoma preoperatively. Therefore, FNA (fine needle aspiration) and needle biopsy are not indicated before the surgery as they pose the risk of tumor dissemination. Hence FNA can be performed under the guidance of EUS (endoscopic ultrasound).
What Are the Differential Diagnosis of Biliary Cystadenoma?
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Hydatid cyst - A cyst occurs by a parasitic infection most commonly seen in the liver and lungs.
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Hematomas - Abnormal collection of blood outside the blood vessels.
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Liver abscess - A pus-filled mass in the liver.
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Post-traumatic cyst - A rare consequence of severe soft tissue trauma.
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Polycystic liver disease - A medical condition characterized by multiple cysts inside the liver which cause abdominal distension.
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Choledochal cyst - A congenital anomaly of the tube that communicates the liver and gall bladder.
What Are the Complications of Biliary Cystadenoma?
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Bleeding.
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Obstructive jaundice - A type of jaundice that occurs from narrowing or obstruction of the bile duct, which prevents drainage of bile from the blood into the intestine.
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Infection.
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Rupture.
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Malignant transformation.
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Ascites - A disease characterized by an abnormal fluid collection inside the abdominal cavity.
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Inferior vena cava obstruction - An abnormal compression or obstruction of the inferior vena cava results in decreased venous return to the heart.
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Gastric outlet obstruction - A condition in which obstruction of the stomach or intestine results in vomiting after food (postprandial), abdominal pain, and weight loss.
What Are the Treatment Options for Biliary Cystadenoma and Cystadenocarcinoma?
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Due to the lack of reliability in imaging and differentiating biliary cystadenoma from cyst adenocarcinoma, the suspected biliary cystadenoma needs to be surgically removed along with the negative margin.
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If the biliary cystadenoma is large and confined to a few segments, it can be treated with hepatectomy. However, some procedures such as fenestration, aspiration, sclerotherapy, internal drainage, and partial resection are limited as they risk a high recurrence rate ranging from 90 to 100 percent.
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Cyst enucleation can be performed when the resection is difficult or associated with morbidity.
Conclusion:
Biliary cystadenoma affects about 90 percent of females aged 45 years more than males, while biliary cystadenocarcinoma affects both men and women equally. MRCP (magnetic resonance cholangiopancreatography) helps visualize cystic communication with the biliary tree and to identify the internal septations. The prognosis of biliary cystadenoma is good if the complete excision of the lesion is performed. Even the prognosis of cystadenocarcinoma is good as they are less aggressive, slow-growing, and less commonly metastasis than other malignancies. Even though the patient is asymptomatic and suspected of having biliary cystadenoma, surgery is required.