Cholangiocarcinoma complicating recurrent primary sclerosing cholangitis after liver transplantation

Khorsandi, Shirin Elizabeth; Salvans, Sílvia; Zen, Yoh; Agarwal, Kosh; Jassem, Wayel; Heaton, Nigel

doi:10.1111/j.1432-2277.2011.01324.x

Cited by 20 publications

(4 citation statements)

References 22 publications

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“…In up to 30%-42% of cases, PSC-associated CCA is found incidentally, on autopsy or liver explants following OLT[ 76 , 77 ]. CCA may even develop de novo in the liver following OLT[ 78 - 80 ], or in the native common bile duct (CBD), when this has been preserved[ 4 , 81 , 82 ]. CCA occurs in up to 20% of patients with post-transplant recurrent PSC[ 83 , 84 ].…”

Section: Incidence and Prevalencementioning

confidence: 99%

Review of incidence and outcomes of treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis

Saffioti

Mavroeidis

2021

WJGO

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Primary sclerosing cholangitis (PSC) is a premalignant condition and a well-documented risk factor for cholangiocarcinoma (CCA) which is the most common malignancy in this setting and the leading cause of deaths in the recent years, with an increasing incidence. PSC-associated CCA has a geographical distribution that follows the incidence of PSC, with an observed ascending gradient from the Eastern to the Western and from the Southern to the Northern countries. It may arise at any location along the biliary tree but is most common in the perihilar area. Patients with PSC and intrahepatic or perihilar CCA are typically not suitable for liver resection, which is otherwise the treatment of choice with curative intent in patients with resectable tumours, providing a radical resection with clear margins can be achieved. This largely relates to the commonly advanced stage of liver disease at presentation, which allows consideration for liver resection only for a very limited number of suitable patients with PSC. On the other hand, remarkable progress has been reached in the last decades with the implementation of a protocol combining neoadjuvant chemoradiation and orthotopic liver transplantation (OLT) for the treatment of perihilar CCA, within specific criteria. Excellent results have been achieved particularly for PSC patients with this cancer, who seem to benefit the most from this treatment, having converted this into an accepted indication for transplantation and the standard of care in several experienced centres. Intrahepatic CCA as an indication for OLT remains controversial and has not been accepted given disappointing previous results. However, as recent studies have shown favourable outcomes in early intrahepatic CCA, it may be that under defined criteria, OLT may play a more prominent role in the future. Distal CCA in the context of PSC requires aggressive surgical treatment with curative intent, when feasible. This review provides insight about particular features of CCA in the setting of PSC, with a main focus on its incidence, considerations relating to its anatomical location and implications to treatment and outcomes, through the viewpoint of historical evolution of management, and future perspectives.

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Section: Incidence and Prevalencementioning

confidence: 99%

Review of incidence and outcomes of treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis

Saffioti

Mavroeidis

2021

WJGO

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“…Considering the incidence of rPSC, de novo CCA after LT is surprisingly rare. With only two reported cases of de novo CCA in the setting of rPSC after LT, 96,97 it is difficult to draw any conclusions on the pathophysiology, but the rarity of de novo CCA has been theorized to be related to the immunosuppression, altering the microenvironment in the setting of chronic inflammation that precedes dysplastic carcinoma transformation. 98 Although screening for CCA with regularly scheduled cross-sectional imaging with ultrasound or MRCP and serial CA 19-9 measures is encouraged by consensus recommendations for all patients with PSC, 99,100 there are no data to support similar practices in the post-LT setting.…”

Section: Malignancy After Liver Transplantationmentioning

confidence: 99%

Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

Martin

Levy

2017

Semin Liver Dis

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Primary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.

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“…In addition, patients with primary sclerosing cholangitis who undergo orthotopic liver transplantation may develop cholangiocarcinoma at follow-up surveillance (72). At imaging, recurrent cholangiocarcinoma can manifest as multifocal hepatic masses, focal bile duct wall thickening with stricture formation, and perihilar masses (Fig 17) (73).…”

Section: Cholangiocarcinomamentioning

confidence: 99%

Malignancy after Solid Organ Transplantation: Comprehensive Imaging Review

Katabathina¹,

Menias²,

Tammisetti³

et al. 2016

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Life expectancies for solid organ recipients as well as graft survival rates for these patients have improved over the years because of advanced immunosuppressive therapies; however, with chronic use of these drugs, posttransplant malignancy has become one of the leading causes of morbidity for them. The risk of carcinogenesis in transplant recipients is significantly higher than for the general population and cancers tend to manifest at an advanced stage. Posttransplant malignancies are thought to develop by three mechanisms: de novo development, donor-related transmission, and recurrence of a recipient's pretransplant malignancy. Although nonmelanoma skin cancer, Kaposi sarcoma, posttransplant lymphoproliferative disorder, anogenital cancer, and lung cancer are malignancies that are thought to arise de novo, malignant melanoma and cancers that arise in the renal allograft are frequently donor related. Hepatocellular carcinomas and cholangiocarcinomas have a greater tendency to recur in liver transplant recipients. An altered or deranged immune system caused by chronic immunosuppression is considered to be one of the major contributing factors to carcinogenesis. The proposed pathogenic mechanisms for oncogenesis include impaired immunosurveillance of neoplastic cells, weakened immune activity against oncogenic viruses, and direct carcinogenic effects of immunosuppressive agents. Imaging plays an important role in screening, follow-up, and long-term surveillance in patients with malignancies because key imaging features can guide in their timely diagnosis. However, some benign entities such as transplant-related renal fibrosis, biliary necrosis, and infectious nodules in the lungs mimic malignancies and require pathologic confirmation. Management strategies that can improve malignancy-related morbidity and mortality in transplant recipients include prevention of risk factors, appropriate modulation of immunosuppressive agents, prophylaxis against infection-related malignancies, and use of intensive targeted screening programs. (©)RSNA, 2016.

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Cholangiocarcinoma complicating recurrent primary sclerosing cholangitis after liver transplantation

Cited by 20 publications

References 22 publications

Review of incidence and outcomes of treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis

Review of incidence and outcomes of treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis

Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

Malignancy after Solid Organ Transplantation: Comprehensive Imaging Review

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