Intrahepatic cholangiocarcinoma in hepatolithiasis: A frequently overlooked disease

Sheen-Chen, Shyr Ming; Chou, Fong‐Fu; Eng, Hock‐Liew

doi:10.1002/jso.2930470213

Cited by 78 publications

(34 citation statements)

References 11 publications

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“…Hepatolithiasis are gallstones located in the intrahepatic bile ducts, which is an established risk factor for CCA. The incidence of IH-CCA in patients with hepatolithiasis is reported to be 4-11% [39]. CCA development in patients with hepatolithiasis is very likely caused by recurrent cholangitis and chronic inflammation of the biliary epithelium [40].…”

Section: Cholelithiasismentioning

confidence: 99%

Epidemiology and Risk Factors of Cholangiocarcinoma

Kirstein

Vogel²

2016

Visc Med

150

127

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Background: Cholangiocarcinoma (CCA) is the second most common primary liver cancer, being characterized by its late diagnosis and fatal outcome. Recent epidemiological reports indicate an increasing worldwide incidence of intrahepatic CCA but a decreasing incidence of extrahepatic CCA. Methods: In this review, we present an overview of the incidence and epidemiology of CCA and possible strategies for screening and surveillance. Results: Efficient strategies for the screening and surveillance of CCA have not been established so far. The vast majority of CCA occur sporadically without any apparent cause; however, several risk factors such as liver flukes, chronic biliary and liver diseases, and lifestyle-related aspects causing chronic inflammation and cholestasis in the liver have been linked to the development of CCA. These risk factors likely contribute to the increased incidence observed in some countries and also explain the wide geographical differences in the incidence of CCA. Conclusion: Several risk factors for CCA have been identified. Given the dismal prognosis of advanced CCA, regular surveillance examinations with a combination of ultrasonography and laboratory tests appear to be useful in patients at risk and need to be explored in prospective trials.

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Section: Cholelithiasismentioning

confidence: 99%

Epidemiology and Risk Factors of Cholangiocarcinoma

Kirstein

Vogel²

2016

Visc Med

150

127

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“…9 However, in cholangiocellular carcinoma some drawbacks of ERCP are known. 10,11 As ERCP was non-diagnostic an intrahepatic nature of the icterus was assumed necessitating a histologic diagnosis. A transjugular biopsy was performed because this method has proved to be a useful technique particularly in patients after BMT 12 because of minimising the bleeding hazard due to thrombocytopenia.…”

Section: Discussionmentioning

confidence: 99%

Bile duct adenocarcinoma mimicking veno-occlusive disease after autologous bone marrow transplantation for acute leukaemia

Becherer

Schulenburg

et al. 1998

Bone Marrow Transplant

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Summary:A 52-year-old female underwent autologous BMT because of acute myeloid leukaemia FAB M4 in second remission. Since the patient had no HLA-identical sibling she received a purged autologous BM transplant. On day +5 she developed signs of a sepsis syndrome with fluid retention and was treated with broad-spectrum antibiotic therapy. However, her body weight remained high, ascites and an increase of total serum bilirubin and alkaline phosphatase developed. The icterus worsened to a total bilirubin level of 25 mg/100 ml. Sonographic and endoscopic imaging showed a dilated gall bladder but disclosed a post-hepatic cause for the icterus. A transjugular liver biopsy on day +71 revealed severe cholestasis and siderosis. The patient remained aplastic with constantly increased bilirubin levels. On day +73 septic shock syndrome occurred and the patient died of multiorgan failure 3 days later. At autopsy, a highly differentiated bile duct adenocarcinoma at the porta hepatis, so-called Klatskin tumour, was found, explaining the fatal course with intractable cholestasis. Keywords: hepatic VOD; cholestasis; bile duct adenocarcinoma Hepatic veno-occlusive disease (VOD) is a syndrome consisting of weight gain, ascites, increase of bilirubin level, and right upper abdominal quadrant pain with a high rate of lethality. Occasionally it can be accompanied by refractoriness to platelet transfusion. In most cases VOD occurs after allogeneic BMT, and occasionally after high-dose chemotherapy with or without autologous stem cell rescue. Despite a range of prophylactic and therapeutic approaches with administration of heparin and tissue plasminogen activator (tPA), 1 transjugular intrahepatic portosystemic stent- shunt (TIPS) 2-4 and even liver transplantation 5 the prognosis has remained poor.We describe a patient who presented clinically with the signs of severe VOD after autologous BMT for AML with an atypical chronic course and a surprising diagnosis post mortem. Case reportA 52-year-old female patient was referred to our department for BMT. She had AML FAB M4 in second remission. The disease had been diagnosed 16 months earlier and the patient had received induction therapy consisting of DNR, vepeside and Ara-C. Complete remission was achieved. Consolidation therapy with Ara-C followed. Two months after the third consolidation cycle the patient had a relapse. She received reinduction therapy with idarubicin and Ara-C and one course of consolidation therapy prior to BMT. Because the patient had no HLA-identical sibling, autologous BM for transplantation was harvested in second remission 2 months before BMT. The BM was purged by mafosfamide. The CD34 + count after purging was 1.44 × 10 6 /kg. On admission for autologous BMT the patient presented with a Karnofsky score of 100. Clinical examination revealed no pathologic findings. The liver and kidney function was normal.Conditioning consisted of BU 4 mg/kg orally daily divided into four doses, total dose 16 mg/kg and vepeside 60 mg/kg. The patient tolerated the conditioning regi...

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“…This highlights the problem of the concomitant occurrance of intrahepatic stones with cholangiocarcinoma. It has been reported that, in 5.0-8.7% [16][17][18] of the patients, intrahepatic stones were concomitant with cholangiocarcinoma. Chronic prolifer ative cholangitis in the presence of intrahepatic stones could undergo progressive changes to atypical epithelial hyperplasia, which might in turn progress to cholangio carcinoma [19].…”

Section: Discussionmentioning

confidence: 99%

Long-Term Results of One Hundred and Seven Hepatic Resections for Intrahepatic Stones

et al. 1992

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During 11 years, 107 of 586 patients (18.3%) with intrahepatic stones underwent hepatic resection to remove intrahepatic stones at Kaohsiung Medical College Hospital. Guided by the location of the intrahepatic stones, 80 (74.8%) of these patients underwent left lateral segmentectomy, 21 (19.6%) left lobectomy, and 6 (5.6%) right posterior segmentectomy. There were no operative mortalities. The postoperative complications were usually minor: wound infection occurred in 21 patients (19.6%); incisional hernia in 2 (1.9%); adhesive ileus in 2 (1.9%); pleural effusion in 2 (1.9%), and subphrenic abscess in 2 (1.9%). Ten patients (9.3%) had residual stones which were later removed by postoperative choledochoscopy. The mean number of postoperative choledochoscopy sessions was 1.29 ± 1.44, which was signiñcantly lower than 10.1 ± 6.7 for those not treated with hepatic resection for their intrahepatic stones (p < 0.001). In the long-term follow-up (median 3 years and 2 months) of 96 patients, 1 patient developed cholangiocarcinoma in a resected stump of the left intrahepatic duct and 4 patients (4.2%) had recurrent stones. The incidence of recurrent stones was also significantly lower than that of patients without hepatic resection: 4.2 vs. 23.8% (p < 0.001). This leads us to conclude that hepatic resection is the treatment of choice for intrahepatic stones because it carries no operative mortality and has a low postoperative morbidity. Virtually all intrahepatic stones can be removed in an operation which is able to eradicate the stenotic ducts to prevent recurrent stones in the long term.

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Intrahepatic cholangiocarcinoma in hepatolithiasis: A frequently overlooked disease

Cited by 78 publications

References 11 publications

Epidemiology and Risk Factors of Cholangiocarcinoma

Epidemiology and Risk Factors of Cholangiocarcinoma

Bile duct adenocarcinoma mimicking veno-occlusive disease after autologous bone marrow transplantation for acute leukaemia

Long-Term Results of One Hundred and Seven Hepatic Resections for Intrahepatic Stones

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