Intrahepatic cholangiocarcinoma after Fontan procedure in an adult with visceral heterotaxy

Wang, Dehua; Marshall, Darren; Veldtman, Gruschen; Gupta, Anita; Trout, Andrew T.; Villafañe, Juan; Bove, Kevin E.

doi:10.1016/j.prp.2018.03.016

Cited by 10 publications

(9 citation statements)

References 18 publications

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“…8 Congestive hepatopathy is the initial and primary manifestation followed by the development of fibrosis, but focal liver lesions inclusive of benign nodules and cancers, most often hepatocellular carcinoma, are being increasingly reported. 6 The…”

Section: Discussionmentioning

confidence: 99%

“…This case has been previously reported. 6 Posttransplant lymphoproliferative disorder (PTLD) was confirmed in one of the patients with history of cardiac transplant and is suspected in the other posttransplant patient. One lesion that appeared distinct by imaging (7%) was diagnosed as vascularized scar tissue by histopathology.…”

Section: Histopathology Of Liver Lesionsmentioning

confidence: 99%

“…Lesions that have been described in patients post-Fontan include hepatocellular carcinoma, hepatocellular adenomas (rare), intrahepatic cholangiocarcinoma, and most often regenerative and focal nodular hyperplasia (FNH)-like hypervascular nodules. 2,[4][5][6] The purpose of this study was to add to the existing literature regarding focal liver lesions in patients after the Fontan operation by reporting our single-center experience with biopsy-proven focal liver lesions in this population. We have deliberately limited this report to lesions diagnosed on the basis of histopathologic sampling, as liver lesions in this population may have atypical imaging appearances which can lead to incorrect diagnoses.…”

Section: Introductionmentioning

confidence: 99%

“…Increasingly, focal liver lesions, including hepatocellular neoplasms, are being identified in Fontan patients. Lesions that have been described in patients post‐Fontan include hepatocellular carcinoma, hepatocellular adenomas (rare), intrahepatic cholangiocarcinoma, and most often regenerative and focal nodular hyperplasia (FNH)‐like hypervascular nodules …”

Section: Introductionmentioning

confidence: 99%

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Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology‐pathology case series

Engelhardt

Trout

Sheridan

et al. 2018

Congenital Heart Disease

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Purpose Patients who have undergone Fontan palliation of single ventricle physiology congenital heart disease are prone to developing focal liver lesions. In our experience, the variety of lesions occurring in this population is greater than that described in the literature. The purpose of this study was to describe the breadth of biopsy‐proven liver lesions in patients post–Fontan palliation of single ventricle physiology cared for at our institution. Methods We retrospectively identified patients who had previously undergone the Fontan operation and had a focal liver lesion biopsied between January 2000 and June 2018. Medical records were reviewed for lesion pathology, background liver findings, pertinent laboratory data, and demographic data. CT and MRI images were reviewed to describe imaging findings of the reported lesions. Results Twelve patients met inclusion criteria; 58% (7/12) of which were female. Fifteen lesions were biopsied including four macroregenerative/benign hepatocellular hyperplastic nodules, two hepatocellular adenomas, two hepatocellular carcinomas, two intrahepatic cholangiocarcinoma (in the same patient), one venous malformation, and one focus of vascularized scar tissue. Two additional lesions in patients postcardiac transplant were posttransplant lymphoproliferative disorder. Conclusion Patients who have undergone Fontan palliation of single ventricle physiology are prone to develop a variety of liver lesions, both benign and malignant.

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Section: Discussionmentioning

confidence: 99%

Section: Histopathology Of Liver Lesionsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology‐pathology case series

Engelhardt

Trout

Sheridan

et al. 2018

Congenital Heart Disease

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“…Na literatura, alguns NH descritos em pacientes após o PF foram biopsiados ou ressecados, com diagnósticos de HNR, HNF, adenoma, CHC e colangiocarcinoma (27,30,32,44,61,62,64,65,68,73,(75)(76)(77)87) . Os nódulos malignos apresentaram dimensões que variaram entre 2,0 e 10,0 cm, o intervalo após o PF variou de 11 a 30 anos, alfafetoproteína não estava elevada em todos os pacientes com CHC e trombose tumoral foi detectada em apenas um caso (32) .…”

Section: Frequência De Nódulos Hepáticosunclassified

Avaliação da frequência de nódulos hepáticos em pacientes submetidos ao procedimento de Fontan: o papel da ultrassonografia, tomografia computadorizada e ressonância magnética

Horvat¹

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MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation

et al. 2018

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Intrahepatic cholangiocarcinoma after Fontan procedure in an adult with visceral heterotaxy

Cited by 10 publications

References 18 publications

Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology‐pathology case series

Focal liver lesions following Fontan palliation of single ventricle physiology: A radiology‐pathology case series

Avaliação da frequência de nódulos hepáticos em pacientes submetidos ao procedimento de Fontan: o papel da ultrassonografia, tomografia computadorizada e ressonância magnética

MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation

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