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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 showing nodular surface with somewhat exophytic mass on right. (c) Microscopic picture of mass, H&E, 20x. (d) Main mass with immunohistochemical stain for CD34 outlining neovascularization. (e) H&E of second “subnodule,” 10x.
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fig2: (a) CT scan of liver. (b) Gross picture showing nodular surface with somewhat exophytic mass on right. (c) Microscopic picture of mass, H&E, 20x. (d) Main mass with immunohistochemical stain for CD34 outlining neovascularization. (e) H&E of second “subnodule,” 10x.

Mentions: A 25-year-old physician underwent a two-step Kasai procedure as an infant, first at 40 days then at 2 years of age. Surgeries were performed in another country. He had not received any surveillance endoscopies for varices since he was about 5 years of age. His clinical course was complicated by splenomegaly, thrombocytopenia, nonbleeding esophageal varices, and hepatic encephalopathy mostly due to the progression to cirrhosis. The varices regressed and his treating physician in his native country believed that he developed shunts that contributed to development of his encephalopathy. A CT liver triple phase with contrast showed a cirrhotic liver with small hypodensity and portal venous phase that persisted on delayed images in the right inferior lobe (Figure 2(a)). The patient underwent liver transplant. Two nodules were seen in the explanted liver, both in segment 7, measuring 2.5 and 1.2 cm in greatest dimension. The nodules were positive for polyclonal CEA, CD10, arginase-1, glypican 3 (focal and granular), CD34 (Figure 2(d)), and beta-catenin (membranous). They were negative for alpha-fetoprotein immunostain. The larger nodule was encapsulated and exophytic in nature (Figure 2(b)). The smaller nodule was a “subnodule” growing within a high-grade dysplastic nodule (Figure 2(e)). They were both diagnosed as well-differentiated hepatocellular carcinoma and staged as pT2pNX. Approximately 2 months after transplant, a liver biopsy was done due to persistently elevated transaminases. However, no acute rejection was seen. The patient returned to his native country shortly thereafter.


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 showing nodular surface with somewhat exophytic mass on right. (c) Microscopic picture of mass, H&E, 20x. (d) Main mass with immunohistochemical stain for CD34 outlining neovascularization. (e) H&E of second “subnodule,” 10x.
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4539070&req=5

fig2: (a) CT scan of liver. (b) Gross picture showing nodular surface with somewhat exophytic mass on right. (c) Microscopic picture of mass, H&E, 20x. (d) Main mass with immunohistochemical stain for CD34 outlining neovascularization. (e) H&E of second “subnodule,” 10x.
Mentions: A 25-year-old physician underwent a two-step Kasai procedure as an infant, first at 40 days then at 2 years of age. Surgeries were performed in another country. He had not received any surveillance endoscopies for varices since he was about 5 years of age. His clinical course was complicated by splenomegaly, thrombocytopenia, nonbleeding esophageal varices, and hepatic encephalopathy mostly due to the progression to cirrhosis. The varices regressed and his treating physician in his native country believed that he developed shunts that contributed to development of his encephalopathy. A CT liver triple phase with contrast showed a cirrhotic liver with small hypodensity and portal venous phase that persisted on delayed images in the right inferior lobe (Figure 2(a)). The patient underwent liver transplant. Two nodules were seen in the explanted liver, both in segment 7, measuring 2.5 and 1.2 cm in greatest dimension. The nodules were positive for polyclonal CEA, CD10, arginase-1, glypican 3 (focal and granular), CD34 (Figure 2(d)), and beta-catenin (membranous). They were negative for alpha-fetoprotein immunostain. The larger nodule was encapsulated and exophytic in nature (Figure 2(b)). The smaller nodule was a “subnodule” growing within a high-grade dysplastic nodule (Figure 2(e)). They were both diagnosed as well-differentiated hepatocellular carcinoma and staged as pT2pNX. Approximately 2 months after transplant, a liver biopsy was done due to persistently elevated transaminases. However, no acute rejection was seen. The patient returned to his native country shortly thereafter.

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