Two Rare Cases of Hepatocellular Carcinoma after Kasai Procedure for Biliary Atresia: A Recommendation for Close Follow-Up

Hirzel, Alicia; Madrazo, Beatrice L.; Rojas, Claudia

doi:10.1155/2015/982679

Cited by 7 publications

(9 citation statements)

References 14 publications

(19 reference statements)

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“…256 Biliary atresia may be predisposed to development of a malignancy albeit rare, even after the child has had a successful Kasai surgery as these children often develop cirrhosis. 257 Patients with progressive familial intrahepatic cholestasis (PFIC) type 2 present with pruritus and jaundice secondary to bile salt export pump deficiency and are at up to 10% risk for development of HCC by 2 years of age. 258 In cholestatic syndromes that may not be associated with cirrhosis like Alagille's, PFIC 1, and PFIC 3, there is low risk of development of a liver malignancy.…”

Section: Etiologymentioning

confidence: 99%

2019 Update of Indian National Association for Study of the Liver Consensus on Prevention, Diagnosis, and Management of Hepatocellular Carcinoma in India: The Puri II Recommendations

Kumar

Acharya

Singh

et al. 2020

Journal of Clinical and Experimental Hepatology

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Hepatocellular carcinoma (HCC) is one of the major causes of morbidity, mortality, and healthcare expenditure in patients with chronic liver disease in India. The Indian National Association for Study of the Liver (INASL) had published its first guidelines on diagnosis and management of HCC (The Puri Recommendations) in 2014, and these guidelines were very well received by the healthcare community involved in diagnosis and management

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Section: Etiologymentioning

confidence: 99%

2019 Update of Indian National Association for Study of the Liver Consensus on Prevention, Diagnosis, and Management of Hepatocellular Carcinoma in India: The Puri II Recommendations

Kumar

Acharya

Singh

et al. 2020

Journal of Clinical and Experimental Hepatology

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“…The discrepancy between imaging and histopathology in the setting of BA has been reported in 32 pediatric cases [ 2 , 3 , 10 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 ]. The prevalence of HCC in explants of 544 children transplanted for BA was reported to be 1.2% [ 3 , 10 , 26 ].…”

Section: Discussionmentioning

confidence: 99%

“…Half of HCC seem to be missed during pre-LT imaging, and 41% of BA patients with HCC have a normal AFP [ 3 ]. The calculated sensitivity for the radiological diagnosis of HCC within the available literature of BA is 62.5% [ 2 , 3 , 10 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 29 , 30 , 31 , 32 , 33 ]. AFP was also of very limited use in our series, since it was only increased in one of the two patients with HCC.…”

Section: Discussionmentioning

confidence: 99%

Features of Nodules in Explants of Children Undergoing Liver Transplantation for Biliary Atresia

Calinescu

Rougemont

Anooshiravani

et al. 2022

JCM

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(1) Background: In patients with biliary atresia (BA) liver nodules can be identified either by pre-transplant imaging or on the explant. This study aimed to (i) analyze the histopathology of liver nodules, and (ii) to correlate histopathology with pretransplant radiological features. (2) Methods: Retrospective analysis of liver nodules in explants of BA patients transplanted in our center (2000–2021). Correlations with pretransplant radiological characteristics, patient age at liver transplantation (LT), time from Kasai hepatoportoenterostomy (KPE) to LT, age at KPE and draining KPE. (3) Results: Of the 63 BA-patients included in the analysis, 27/63 (43%) had nodules on explants. A majority were benign macroregenerative nodules. Premalignant (low-grade and high-grade dysplastic) and malignant (hepatocellular carcinoma) nodules were identified in 6/63 and 2/63 patients, respectively. On pretransplant imaging, only 13/63 (21%) patients had liver nodules, none meeting radiological criteria for malignancy. The occurrence of liver nodules correlated with patient age at LT (p < 0.001), time KPE-LT (p < 0.001) and draining KPE (p = 0.006). (4) Conclusion: In BA patients, pretransplant imaging did not correlate with the presence of liver nodules in explants. Liver nodules were frequent in explanted livers, whereby 25% of explants harboured malignant/pre-malignant nodules, emphasizing the need for careful surveillance in BA children whose clinical course may require LT.

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“…Okuyama et al [5] reported the cases of HCC occurrence after BA surgery in 1965, and thereafter a total of 25 cases have been reported from Japan and overseas [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21], of which 10 were from Japan [6-11, 18, 21].…”

Section: Discussionmentioning

confidence: 99%

“…And, MRI findings finally led to the diagnosis of HCC. Some reports recommend regular follow-up by MRI to ensure the accuracy and to avoid radiation exposure [13,14,16,20]. These facts led us to consider it necessary to accumulate more data on the selection of subject cases (states) and the frequency of performing MRI.…”

Section: Archives Of Clinical and Medical Case Reports 113mentioning

confidence: 99%

Identification of Itch Mediators and Its Novel Pathway: Implications for Understanding Radiation-Induced Skin Effects

Lee¹,

Sun²

2016

Arch Clin Biomed Res

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Here we report a case of long-term survival with own liver in the patient of biliary atresia (BA) who developed hepatocellular carcinoma (HCC). The patient was a 34-year-old man. He underwent a portal jejunostomy with a diagnosis of BA (IIIb1ν) on the 94th day after birth, with a favorable post-operative course. Contrast-enhanced CT upon admission for cholangitis at the age of 34 suspected HCC, with an AFP level of 1.3 ng/ml and increased PIVKAII to 1,199 mAU/ml. He was diagnosed with HCC on MRI, and the tumor embolus had spread to the left branch of the portal vein; thus, resection and transplantation were considered contraindication. Proton therapy was performed, but he died 11 months after the detection of HCC. To date, 26 cases of HCC including this one have been reported, of which 20 were those in minors. Many of the patients with early onset have poor prognosis such as poor reduction of jaundice, while many of those with adult onset have relatively favorable prognosis as in this case; therefore, it was suggested that the progression of cholestatic cirrhosis is associated with carcinogenesis.

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Two Rare Cases of Hepatocellular Carcinoma after Kasai Procedure for Biliary Atresia: A Recommendation for Close Follow-Up

Cited by 7 publications

References 14 publications

2019 Update of Indian National Association for Study of the Liver Consensus on Prevention, Diagnosis, and Management of Hepatocellular Carcinoma in India: The Puri II Recommendations

2019 Update of Indian National Association for Study of the Liver Consensus on Prevention, Diagnosis, and Management of Hepatocellular Carcinoma in India: The Puri II Recommendations

Features of Nodules in Explants of Children Undergoing Liver Transplantation for Biliary Atresia

Identification of Itch Mediators and Its Novel Pathway: Implications for Understanding Radiation-Induced Skin Effects

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