Liver resection and transplantation in Caroli disease and syndrome

Fahrner, René; Dennler, Sandra; Dondorf, Felix; Ardelt, Michael; Rauchfuß, F.; Settmacher, Utz

doi:10.1016/j.jviscsurg.2018.06.001

Cited by 22 publications

(27 citation statements)

References 29 publications

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“…The total incidence of cancerous transformation or simultaneous cancer in CS and CD is approximately 7%, but it ranged from approximately 3% to almost 40% in the beginning of the 2000s depending on the cohort[ 19 , 27 ]. Over time this incidence did not change, and the latest publications during the last six years reported incidences between 5% to 33%[ 12 , 20 , 25 ]. There were no new aspects reported on the development of cancerous transformation, and biliary stasis[ 8 ], stone irritation[ 10 ], a cellular predisposition of the cystic congenital malformation[ 8 ] and a combination of these factors are possible pathways[ 28 ].…”

Section: Discussionmentioning

confidence: 99%

“…The tumor markers Ca-19-9 is often elevated in cases of benign biliary obstruction or cholangitis, and higher levels may mislead the correct diagnosis[ 7 ]. Overall, the diagnosis of malignancy in CS and CD is difficult, and the tumor identification was an incidental finding in most of the reports in this review, which was made during the final histological assessment of the surgical specimen, because the preoperative identification of a tumor within a dilated cystic bile tract is almost impossible[ 3 , 8 , 11 , 13 , 25 ]. Therefore, the value of screening patients with CS and CD for malignancy is quite inaccurate, and definite treatment should be sought, because delayed diagnosis and widespread tumor are associated with a poor prognosis[ 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…As a conclusion for this observation, surgical treatment with liver resection or OLT should be offered to patients with CS or CD as soon as possible to avoid recurrent sepsis and primarily malignant transformation[ 3 , 25 , 30 ]. In cases of conservative treatment, even asymptomatic patients should be regularly screened clinically and radiologically for malignancy[ 30 ].…”

Section: Discussionmentioning

confidence: 99%

“…The complaint symptoms may be unspecific and vary from pain, pruritus, fever, sepsis, recurrent cholangitis, abscesses, pancreatitis, weight loss, and adynamia[ 3 , 5 , 15 - 22 ]. Besides biliary drainage operations or interventions[ 7 , 22 - 24 ], liver resection[ 7 , 12 , 25 ] or orthotopic liver transplantation (OLT)[ 7 ] are performed. The decision to perform a specific intervention depends on the extent of biliary pathology and hepatic fibrosis and portal hypertension consecutively.…”

Section: Introductionmentioning

confidence: 99%

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Risk of malignancy in Caroli disease and syndrome: A systematic review

Fahrner¹,

Dennler²,

Inderbitzin³

2020

WJG

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BACKGROUND Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease (CD), and is called Caroli syndrome (CS) when it has fibrotic and cirrhotic liver morphology. The development of intrahepatic carcinoma is described in both conditions, but the reported incidence varies extensively. Potential risk factors for the malignant transformation were not described. Furthermore, conservative or surgical treatment is performed depending on the extent of cystic malformation, hepatic dysfunction and structural hepatic changes, but little is known about which treatment should be offered to patients with CD or CS and cancer. AIM To further investigate the malignant transformation in these conditions. METHODS A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. A search using Medline (PubMed) was performed using a combination of Medical Subject Headings terms “caroli disease”, “caroli syndrome”, “tumor”, “malignant”, and “cholangiocarcinoma”. Only human studies published in English were used for this systematic review. The following parameters were extracted from each article: year of publication, type of study, number of patients, incidence of malignant tumor, duration of symptoms, age, sex, diagnostics, identification of tumor, surgical therapy, survival and tumor recurrence. RESULTS Twelve retrospective studies reporting the courses of 561 patients (53% females) were included in this systematic review. With a mean age of 41.6 years old (range 23 to 56 years old), patients were younger than other populations undergoing liver surgery. Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7% to 37.5% with an overall incidence of 6.6%. There were only few detailed reports about preoperative diagnostic work-up, but a multimodal work-up including ultrasound of the liver, computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies. Disease duration was variable with up to several years. Most patients had episodes of cholangitis, sepsis, fever or abdominal pain. Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics. Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function. No postoperative adjuvant chemotherapy was reported, but chemotherapy was administered in selected cases of tumor recurrence. Overall survival rates after one year were low at 36% and a high recurrence rate of up to 75% during the observation period. CONCLUSION Only few retrospective studies reported a low tumor incidence. Despite the high rate of...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Risk of malignancy in Caroli disease and syndrome: A systematic review

Fahrner¹,

Dennler²,

Inderbitzin³

2020

WJG

View full text Add to dashboard Cite

show abstract

“…Os tratamentos atualmente disponíveis ainda não oferecem a cura para todos portadores da DC, com boa parte dos pacientes em tratamento ainda relatando períodos de recuperação transitórios, com retorno da sintomatologia, o que os leva a múltiplas internações hospitalares e diminuição da qualidade de vida. (FAHRNER et al, 2019). O tratamento farmacológico mais recomendado é antibioticoterapia específica nos casos de colangite e ácido ursodesoxicólico para litólise nos casos de colelitíase intrahepática (TAYLOR, PALMER, 1998 19,36,97,98,99,100,103,105,106,108,109,110,111,118,119 Diagnóstico 1,5,25,31,38,40,43,46,52,53,58,59,61,62,72,73,79,80,84,85,87,88,90,91,109,112,113,114,115,116,117,120,122,128,130,131,132,…”

Section: Discussionunclassified