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Biliary Cystadenoma

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.


Presentation

Biliary cystadenoma is a cystic (either unilocular or multilocular) intrahepatic lesion that is considered to be a rare entity in clinical practice [1] [2] [3]. The tumor originates in the bile ducts, with the right lobe identified as the principal location in more than half of all cases [1] [4]. However, extrahepatic location (the cystic, common hepatic, and bile ducts) has been documented in a small number of patients [1] [5]. The exact pathogenesis model remains unknown; for some reason, biliary cystadenomas are mainly encountered in middle-aged women [2] [3] [4] [6]. The potential for malignant transformation of biliary cystadenoma is low [7], 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 [3]. 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 [2] [3] [5] [6]. Anorexia, nausea, vomiting, and either weight gain or weight loss are other important symptoms [3] [6]. At some point, biliary tract obstruction leads to jaundice [1] [5] [6].

Soft Tissue Mass
  • The presence of mural or septal nodules, discrete soft-tissue masses, and possibly thick and coarse calcifications increase the likelihood of a cystadenocarcinoma.[ncbi.nlm.nih.gov]
Weight Gain
  • Anorexia, nausea, vomiting, and either weight gain or weight loss are other important symptoms. At some point, biliary tract obstruction leads to jaundice.[symptoma.com]
Swelling
  • Department of General and Gastrointestinal Surgery, Hospital Marqués de Valdecilla, Santander, Cantabria, Spain. [email protected] Abstract We report a case of a large biliary cystadenoma occupying the entire peritoneal cavity, manifesting as an abdominal swelling[ncbi.nlm.nih.gov]
Epigastric Mass
  • A 27-year-old woman was admitted to our hospital with a firm epigastric mass. At laparotomy, a large cystic mass was resected from the left hepatic lobe, and diagnosed as biliary cystadenoma.[ncbi.nlm.nih.gov]
Hepatic Mass
  • We suggest that this obscure entity be considered in the differential diagnosis of cystic hepatic masses, with or without calcific margins.[ncbi.nlm.nih.gov]
  • Abstract A 24-year-old white woman presented with abdominal discomfort associated with a firm, nonpulsatile hepatic mass.[ncbi.nlm.nih.gov]
Suggestibility
  • We suggest that this obscure entity be considered in the differential diagnosis of cystic hepatic masses, with or without calcific margins.[ncbi.nlm.nih.gov]
  • The preoperative diagnosis of biliary cystadenoma and cystadenocarcinoma was suggested due to the radiological detection of vascularity in the septa and the invasion of the thoracoabdominal wall. The surgeon was informed in both of the cases.[ncbi.nlm.nih.gov]
  • The images clearly demonstrate the communication between the multiloculated cystic mass and the biliary tree, suggesting the possibility of biliary cystadenoma. Due to the malignant potential of a cystadenoma, the lesion was resected.[ncbi.nlm.nih.gov]
  • The presence of an enhanced mural nodule is the most important finding to suggest BCAC.[ncbi.nlm.nih.gov]
  • A mural or septal nodule and a nodule diameter greater than 1.0 cm on conventional ultrasound are suggestive of cystadenocarcinomas.[ncbi.nlm.nih.gov]

Workup

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 [7]. 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 [1]. 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) [1] [2] [3] [5] [6], 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 [1] [7] [8]. 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 [5] [8].

Multilocular Cyst
  • Radiological examination, which included radionuclide liver scan, abdominal ultrasonography, computed tomography, and hepatic angiography, demonstrated a large, globular, multilocular cyst arising from the left lobe of the liver.[ncbi.nlm.nih.gov]
  • Computed tomography and abdominal sonography showed characteristic multilocular cysts in the left lobe of the liver. Serum CA 19-9 was elevated to 108 U/ml with normal carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) levels.[ncbi.nlm.nih.gov]
  • 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.[symptoma.com]
  • Ultrasound At US, a biliary cystadenoma appears as a unilocular or multilocular cyst with enhanced through transmission.[radiopaedia.org]

Treatment

  • 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]
  • The mainstay of treatment is complete resection, either by enucleation or by formal hepatectomy, since incomplete removal entails not only constant recurrence but also the risk of malignant transformation to cystadenocarcinoma.[ncbi.nlm.nih.gov]
  • We report the course of 15 patients who underwent resection for biliary cystadenoma to elucidate the clinical presentation, preoperative evaluation, and surgical treatment.[ncbi.nlm.nih.gov]
  • The treatment of choice is surgical excision. It is important to differentiate BCA from HSCs because they have different clinical significances and treatment plans. However, it is difficult to preoperatively differentiate a BCA from a HSC.[ncbi.nlm.nih.gov]
  • Radical resection is the best treatment for IHBCA and can effectively prevent recurrence.[ncbi.nlm.nih.gov]

Prognosis

  • Abstract Biliary cystadenoma and cystadenocarcinoma are rare tumours which have a good prognosis after complete surgical removal. Correct pre-operative diagnosis depends on the imaging characteristics of the tumours.[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 prognosis of biliary cystadenoma after complete resection is excellent. Nevertheless, there is a risk of recurrence or malignant transformation after incomplete resection.[ncbi.nlm.nih.gov]
  • The aims of this study were to characterize the clinical and pathologic aspects of these lesions, and also to discuss the treatment and prognosis of them.[ncbi.nlm.nih.gov]
  • Prognosis is excellent after complete resection.[ncbi.nlm.nih.gov]

Etiology

  • 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]
  • Although the precise etiology of this woman's biliary cystadenoma cannot be ascertained, the high estrogen receptor content in her cystadenoma suggests that these tumors are sensitive to estrogen and that estrogen-containing OCs may serve as tumor promoters[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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Hepatic cystadenomas appear as multilocular (rarely unilocular) cystic lesions that are surrounded by a smooth and thick fibrous capsule.[emedicine.medscape.com]
  • Anatomy and pathophysiology of gastro-esophageal junction (GEJ) in gastro-esophageal prolapse (GEP).[unibo.it]

Prevention

  • 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]

References

Article

  1. 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.
  2. Horton KM, Bluemke DA, Hruban RH, Soyer P, Fishman EK. CT and MR imaging of benign hepatic and biliary tumors. Radiographics. 1999;19(2):431-451.
  3. Thomas KT, Welch D, Trueblood A, et al. Effective Treatment of Biliary Cystadenoma. Ann Surg. 2005;241(5):769-775.
  4. Mortelé KJ, Ros PR. Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 2001;21(4):895-910.
  5. Murphy BJ, Casillas J, Ros PR, et al. The CT appearance of cystic masses of the liver. Radiographics 1989; 9:307-322.
  6. Ariff A, Hassan H, John G. Biliary Cystadenoma - Computed Tomography Findings. Malays J Med Sci. 2002;9(1):49-51.
  7. 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.
  8. 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.

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Last updated: 2018-06-22 04:30