Biliary cystadenoma is a rare benign intrahepatic tumor that arises from the biliary system and is primarily diagnosed in middle-aged women. The clinical presentation includes abdominal pain, nausea, vomiting, and a palpable mass accompanied by jaundice. Symptoms may last for months or years before the diagnosis is made, which increases the risk for rare, but possible malignant transformation. Computed tomography (CT), magnetic resonance imaging (MR), and magnetic resonance cholangiopancreatography (MRCP) are recommended imaging studies.
Biliary cystadenoma is a cystic (either unilocular or multilocular) intrahepatic lesion that is considered to be a rare entity in clinical practice   . The tumor originates in the bile ducts, with the right lobe identified as the principal location in more than half of all cases  . However, extrahepatic location (the cystic, common hepatic, and bile ducts) has been documented in a small number of patients  . The exact pathogenesis model remains unknown; for some reason, biliary cystadenomas are mainly encountered in middle-aged women    . The potential for malignant transformation of biliary cystadenoma is low , but a delay in diagnosis (signs and symptoms can be present up to several years) is perhaps one of the main factors for a poorer prognosis, implying that early recognition is essential . The clinical presentation starts with symptoms such as abdominal pain that is often chronic in nature and a palpable mass in the right upper quadrant that persists for weeks, months, or even years    . Anorexia, nausea, vomiting, and either weight gain or weight loss are other important symptoms  . At some point, biliary tract obstruction leads to jaundice   .
Entire Body System
No ascites. No enlarged node in portal hepatic and para-aortic region. Thin peripheral enhancement. Management: Surgical excision was performed. HPE Caudate lobe lesion of benign serous cystadenoma of the liver. [wsoonli15.wordpress.com]
On CT scans or MRIs, signs of coexistent cirrhosis, such as ascites, macronodules, or hepatomegaly, help clinicians and radiologists differentiate cystic hepatocellular carcinoma from biliary cystadenocarcinoma. [emedicine.medscape.com]
Traditional treatment of simple cysts such as aspiration, drainage, and marsupialization results in near universal recurrence and occasional malignant degeneration. [ncbi.nlm.nih.gov]
Aspiration or partial excision is associated with higher recurrence rate. [synapse.koreamed.org]
Once the diagnosis is confirmed by immunologic testing, these cysts should be treated with percutaneous aspiration, hypertonic saline injection, re-aspiration and drainage (PAIRD) followed by pericystectomy (16). [caribbean.scielo.org]
- Abdominal Distension
Complaints included abdominal pain in 74%, abdominal distension in 26%, and nausea/vomiting in 11%. Only 1 patient presented with an incidental finding. [ncbi.nlm.nih.gov]
However, they often have vague abdominal complaints, abdominal palpable mass, abdominal distension, jaundice, anorexia, nausea or vomiting. [eprints.bice.rm.cnr.it]
The differential diagnosis of biliary cystadenoma is very broad, as hydatid cysts, liver abscesses, other malignant or benign tumors (including its malignant form - biliary cystadenocarcinoma), as well as hemorrhagic cysts, may have a similar clinical and radiologic presentation . For this reason, a meticulous workup should start with a carefully obtained patient history that will assess the course and progression of symptoms, as well as their duration. The physical examination can be a crucial component if a mass is detected during abdominal inspection and palpation. As soon as sufficient evidence suggests a hepatic lesion, imaging studies need to be employed. Abdominal ultrasonography is a useful first-line procedure that is able to reveal typical findings of a biliary cystadenoma - a unilocular or multilocular cyst . More advanced studies, however, are necessary in order to strengthen the diagnosis. Computed tomography (CT) shows a cystic lesion sharply delineated by a thick capsule made of fibrous tissue with internal septation (calcifications may also be present)     , but the introduction of magnetic resonance imaging (MRI, particularly gadolinium-enhanced), and especially magnetic resonance cholangiopancreatography (MRCP) has greatly improved the overall diagnosis rate of many lesions, including biliary cystadenoma   . These two procedures are the gold standard and should be used whenever possible, showing a homogeneously low intensity on T1-weighted images and very high signal intensity on T2-weighted images  .
There remains a need for education about the imaging findings for biliary cystadenoma to reduce the demonstrated delay in appropriate treatment. [ncbi.nlm.nih.gov]
A case of benign biliary cystadenoma with mesenchymal stroma is presented along with a review of the relative literature addressing the clinical presentation, histology, histogenesis, differential diagnosis, imaging features, treatment and prognosis of [ncbi.nlm.nih.gov]
The exact etiology of these tumors is unknown, but several theories have been proposed. Historically these cystic tumors have been treated by a variety of techniques including aspiration, fenestration, internal drainage, and resection. [ncbi.nlm.nih.gov]
TERMINOLOGY Definitions Cystic biliary neoplasm arising within liver May arise in extrahepatic biliary tree, including gallbladder ETIOLOGY/PATHOGENESIS Unknown May arise from gallbladder precursor elements or peribiliary glands Most cystadenocarcinomas [basicmedicalkey.com]
Etiology The etiology of hepatic cystadenomas is unknown. The resemblance of embryonic structures, such as the gallbladder and the biliary tree, originating from the foregut suggests that these lesions arise from ectopic remnants. [emedicine.medscape.com]
Etiology In most cases, adenomas develop for unclear reasons in an otherwise healthy liver. [orpha.net]
The true etiology of IHBCA remains idiopathic since first reported by Keen in 1892 ( 2 ). Abnormal embryonic development resulting in ectopic foregut or gonadal epithelium sequestered in the liver has been proposed ( 3, 4 ). [academic.oup.com]
Clinical presentation, possible origin, epidemiology, and differential diagnosis of biliary cystadenoma and cystadenocarcinoma are discussed. [ncbi.nlm.nih.gov]
Unknown May arise from gallbladder precursor elements or peribiliary glands Most cystadenocarcinomas arise from preexisting biliary cystadenoma Some may represent cystic variant of cholangiocarcinoma Occasionally may arise in biliary cysts CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
Summary Epidemiology Annual incidence is estimated at one case per million. Clinical description Mean age at diagnosis is 34 years (ranging from 15 to 64 years). HA rarely occurs in children. [orpha.net]
Archives of Photogrammetry, Remote Sensing and Spatial …, 2015 1 2015 A new post vulvectomy reconstructive technique FM Camacho, J Navarro, JC Moreno, MA Muñoz Journal of the European Academy of Dermatology and Venereology 8 (2), 137-139, 1997 1 1997 Epidemiology [scholar.google.es]
Epidemiology United States data The prevalence of hepatic cystadenomas is low, with fewer than 200 cases reported in the literature. These tumors account for a very small number of all hepatic tumors. [emedicine.medscape.com]
Radical resection is the best treatment for IHBCA and can effectively prevent recurrence. [ncbi.nlm.nih.gov]
Hence, a high degree of suspicion and a multidisciplinary approach are needed to plan the surgical procedure and prevent inadequate treatment. The surgical strategy would be to radically excise the lesion. [jmedicalcasereports.biomedcentral.com]
- Levy AD, Murakata LA, Abbott RM, Rohrmann CA Jr. From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 2002;22(2):387-413.
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- Billington PD, Prescott RJ, Lapsia S. Diagnosis of a biliary cystadenoma demonstrating communication with the biliary system by MRI using a hepatocyte-specific contrast agent. Br J Radiol. 2012;85(1010):e035-e036.
- Lewin M, Mourra N, Honigman I et-al. Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur Radiol. 2006;16 (2): 407-413.