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

Hirzel AC, Madrazo B, Rojas CP - Case Rep Pathol (2015)

Bottom Line: Among them is the development of hepatocellular carcinoma.We present two cases of hepatocellular carcinoma, after Kasai procedure, from two different age groups, as a recommendation that these patients should be even more closely monitored.Furthermore, if they are in need of transplant, we recommend that the explanted livers be carefully examined, as the tumor may not have been diagnosed preoperatively.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Jackson Memorial Hospital, University of Miami Miller School of Medicine, 1611 NW 12th Avenue, Holtz Building, Room 2042, Miami, FL 33136, USA.

ABSTRACT
The instigation of the Kasai procedure in infants who are born with biliary atresia has led to increased survival in this population for over half a century. The many complications that arise as a result of biliary atresia led to an early death for most patients. However, the Kasai procedure is not without its own impediments. Among them is the development of hepatocellular carcinoma. We present two cases of hepatocellular carcinoma, after Kasai procedure, from two different age groups, as a recommendation that these patients should be even more closely monitored. Furthermore, if they are in need of transplant, we recommend that the explanted livers be carefully examined, as the tumor may not have been diagnosed preoperatively.

No MeSH data available.


Related in: MedlinePlus

(a) CT scan of liver. (b) Gross picture of liver mass. (c) Microscopic picture of liver mass, H&E, 10x, with trichrome inset showing mass in lower half. (d) High magnification of mass, H&E, 20x.
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fig1: (a) CT scan of liver. (b) Gross picture of liver mass. (c) Microscopic picture of liver mass, H&E, 10x, with trichrome inset showing mass in lower half. (d) High magnification of mass, H&E, 20x.

Mentions: A 4-year-old boy, delivered via C-section, presented with obstructive jaundice, dark urine, and clay-colored stool soon after birth (after an uneventful gestational period). The liver biopsy was interpreted as cirrhotic liver with cholestasis, bile ductular proliferation, and bile lakes, consistent with extrahepatic biliary atresia. He suffered an episode of cholangitis prior to his Kasai procedure, which was then postponed until after treatment. He was then lost to follow-up. At 4 years of age he was taken to the hospital due to an enlarging abdominal mass. Ultrasound and magnetic resonance imaging (MRI) detected two lesions, one in segment II of the liver with a second smaller mass in segment III (Figure 1(a)). Biopsies proved the masses to be hepatocellular carcinoma. Liver transplant was scheduled shortly thereafter. The explanted liver had two nodules in the left lobe, 3.8 cm and 2 cm, seen grossly, which were diagnosed as well-differentiated hepatocellular carcinoma and staged as pT2pNx (Figure 1(b)). Reticulin stain disclosed disruption of the lining around the hepatocytes in the hepatocellular carcinoma. Trichrome also highlighted areas of neoplastic tissue (Figures 1(c) and 1(d)). The patient presented with acute rejection 5 months after transplant and was treated. At 8 months after transplant, a liver biopsy was performed and chronic rejection with distortion and paucity of bile ducts was diagnosed. One year after transplant another biopsy was performed which showed bile duct proliferation with cholestasis, parenchymal inflammation, and portal fibrosis. Immunostain for SV40 was negative as was polymerase chain reaction (PCR) for BK virus. After this last biopsy the patient was again lost to follow-up and has not returned to our institution.


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

Hirzel AC, Madrazo B, Rojas CP - Case Rep Pathol (2015)

(a) CT scan of liver. (b) Gross picture of liver mass. (c) Microscopic picture of liver mass, H&E, 10x, with trichrome inset showing mass in lower half. (d) High magnification of mass, H&E, 20x.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4539070&req=5

fig1: (a) CT scan of liver. (b) Gross picture of liver mass. (c) Microscopic picture of liver mass, H&E, 10x, with trichrome inset showing mass in lower half. (d) High magnification of mass, H&E, 20x.
Mentions: A 4-year-old boy, delivered via C-section, presented with obstructive jaundice, dark urine, and clay-colored stool soon after birth (after an uneventful gestational period). The liver biopsy was interpreted as cirrhotic liver with cholestasis, bile ductular proliferation, and bile lakes, consistent with extrahepatic biliary atresia. He suffered an episode of cholangitis prior to his Kasai procedure, which was then postponed until after treatment. He was then lost to follow-up. At 4 years of age he was taken to the hospital due to an enlarging abdominal mass. Ultrasound and magnetic resonance imaging (MRI) detected two lesions, one in segment II of the liver with a second smaller mass in segment III (Figure 1(a)). Biopsies proved the masses to be hepatocellular carcinoma. Liver transplant was scheduled shortly thereafter. The explanted liver had two nodules in the left lobe, 3.8 cm and 2 cm, seen grossly, which were diagnosed as well-differentiated hepatocellular carcinoma and staged as pT2pNx (Figure 1(b)). Reticulin stain disclosed disruption of the lining around the hepatocytes in the hepatocellular carcinoma. Trichrome also highlighted areas of neoplastic tissue (Figures 1(c) and 1(d)). The patient presented with acute rejection 5 months after transplant and was treated. At 8 months after transplant, a liver biopsy was performed and chronic rejection with distortion and paucity of bile ducts was diagnosed. One year after transplant another biopsy was performed which showed bile duct proliferation with cholestasis, parenchymal inflammation, and portal fibrosis. Immunostain for SV40 was negative as was polymerase chain reaction (PCR) for BK virus. After this last biopsy the patient was again lost to follow-up and has not returned to our institution.

Bottom Line: Among them is the development of hepatocellular carcinoma.We present two cases of hepatocellular carcinoma, after Kasai procedure, from two different age groups, as a recommendation that these patients should be even more closely monitored.Furthermore, if they are in need of transplant, we recommend that the explanted livers be carefully examined, as the tumor may not have been diagnosed preoperatively.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Jackson Memorial Hospital, University of Miami Miller School of Medicine, 1611 NW 12th Avenue, Holtz Building, Room 2042, Miami, FL 33136, USA.

ABSTRACT
The instigation of the Kasai procedure in infants who are born with biliary atresia has led to increased survival in this population for over half a century. The many complications that arise as a result of biliary atresia led to an early death for most patients. However, the Kasai procedure is not without its own impediments. Among them is the development of hepatocellular carcinoma. We present two cases of hepatocellular carcinoma, after Kasai procedure, from two different age groups, as a recommendation that these patients should be even more closely monitored. Furthermore, if they are in need of transplant, we recommend that the explanted livers be carefully examined, as the tumor may not have been diagnosed preoperatively.

No MeSH data available.


Related in: MedlinePlus