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Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Month
Hepatocellular carcinoma in chronic Budd Chiari syndrome
Contributed by: Aparna J K, Zubin Driver, Harshitha Shetty
Introduction:
Budd Chiari syndrome (BCS) is a rare hepatic disease caused by occlusion of hepatic venous outflow. BCS induces chronic liver congestion that leads to hepatomegaly, ascites, leg edema, collateral venous dilatation on the abdomen and portal hypertension. Several studies have suggested that hepatic congestion caused by obstruction of hepatic venous outflow can lead to cirrhosis and hepatocellular carcinoma (HCC) [1].
Here. we present a case of multicentric hepatocellular carcinoma against the background of chronic BCS in a 26-year-old man.
Clinical profile:
A 26-year-old man, first symptomatic in 2002, presented with complaints of jaundice and abdominal distension, which resolved on alternative medications.
In 2004, he presented with haemoptysis. The upper GI scopy showed esophageal varices, for which endoscopic ligation was done. On further evaluation, MRI showed left hepatic vein (LHV) thrombosis. There was underlying protein C deficiency leading to a prothrombotic state. LHV stenting was done in 2007 and he was started on anticoagulation therapy. Two years later, he presented with abdominal pain. On evaluation, blockage of the stent had been diagnosed which was conservatively managed. He was symptomatically better till September 2023, when he presented with three episodes of hematemesis and gradually increasing abdominal distension.
On examination, the patient was pale and icteric with an enlarged spleen. Blood investigations revealed anemia, thrombocytopenia, hypoalbuminemia. There was mils elevation of bilirubin and liver enzymes. AFP and PIVKAA levels were raised.
Radiological investigations:
A curvilinear ultrasound probe placed transversely over the right hypochondrium showed altered echotexture of the liver with surface irregularity. The right and middle hepatic veins were not seen and were replaced by collaterals. There was narrowing of the retrohepatic IVC. A stent was seen in the left hepatic vein which showed no colour flow on Doppler evaluation. A well-defined, encapsulated, hypoechoic lesion 7.6 x 5.3 x 6.4 cm with central vascularity was seen in segment VI, VII. Another lesion of similar morphology and sized 8.7 x 7.2 x 7.3 cm was seen in segments IVa, IVb. There was splenomegaly (16 cm) and moderate ascites. CEUS was performed with 2.5 ml of sulphur hexafluoride. The lesion in segment IVa, IVb showed intense homogenous centripetal arterial enhancement in 25-35 seconds with washout at around 2 minutes. (Fig 1 a,b,c )
Figure 1a: Ultrasonography shows well defined heterogeneously hypoechoic lesion in segment VI of liver
Figure 1b: Intensity vs time graph of contrast enhanced ultrasonography shows centripetal contrast enhancement of the lesion in segment IV with washout on delayed phase. (Green – liver parenchyma, Red – Periphery of the lesion, Yellow – Centre of the lesion)
Figure 1c: Contrast enhanced ultrasonography shows progressive centripetal contrast enhancement of the lesion in segment IV, with delayed washout
CECT of the abdomen showed a cirrhotic liver with multiple variable sized, peripherally enhancing irregular hypodense lesions, in segments IV, VI, VII of the liver. The lesions showed homogenous enhancement on arterial phase, peripheral enhancement on venous phase with a central hypodense area and washout on delayed phase. There were no calcifications or cystic areas in the lesions. A stent was seen in the LHV with non-enhancing hypodense content in the lumen suggestive of thrombosis. The intrahepatic IVC was narrowed and none of three hepatic veins was seen. There were multiple tortuous venous collaterals; splenomegaly and ascites. (Fig 2 )
Figure 2a: Non contrast enhanced CT axial image of the abdomen shows a cirrhotic liver with multiple variable sized, hypodense lesions, in segments IV, VI, VII of the liver
Figure 2b: Contrast enhanced CT axial image of the abdomen in arterial phase: The lesions show homogenous enhancement
Figure 2c: Contrast enhanced CT axial image of the abdomen in venous phase: The lesions show washout with peripheral enhancement and a central hypodense area
MRI of the abdomen showed multifocal, multilobulated, well defined, variable sized lesions with smooth margins. They were iso to hypointense on T1WI, hyperintense on T2WI. They showed homogenous arterial enhancement, with no washout in the venous phase and capsular enhancement in the delayed phase. T1 hypointense and T2 hyperintense central scar was seen in most of the lesions. The lesion in segment VI showed diffusion restriction with corresponding drop on ADC. None of the three hepatic veins was seen (Fig 3 a.b,c,d,e)
Figure 3a: Axial T1 weighted MR image of the abdomen shows multifocal, multilobulated, well defined, variable sized, hypointense lesions with smooth margins
Figure 3b: Axial T2 weighted MR image of the abdomen shows the lesions to be hyperintense
Figure 3c: Diffusion weighted axial images of the abdomen shows the lesion in segment VI to have diffusion restriction with corresponding drop on ADC
Figure 3d: Post contrast T1 weighted axial images of the abdomen in arterial phase shows homogenous arterial enhancement
Figure 3e: Post contrast T1 weighted axial images of the abdomen in delayed phase shows capsular enhancement of the lesions
Radiological diagnosis:
Based on the imaging findings and against the background of chronic BCS, the differential diagnosis considered were:
-Multifocal multicentric HCC
-Focal nodular hyperplasia
Pathological diagnosis:
Sonography guided biopsy of lesion in segment VI was done. Microscopy showed fragmented cores of liver tissue with tumour arranged in pseudo glandular and trabecular pattern. Tumour cells were polygonal with mild to moderate nuclear pleomorphism, increased nuclear: cytoplasmic ratio, irregular nuclear membranes and prominent nucleoli. On immunohistochemistry, the tumour showed patchy, strong granular positivity to HAS and was glypican-3 positive. The above features were suggestive of well to moderately differentiated hepatocellular carcinoma
Figure 4a: Histopathology slide image shows tumour arranged in pseudo glandular and trabecular pattern. Tumour cells are polygonal with mild to moderate nuclear pleomorphism, increased nuclear: cytoplasmic ratio, irregular nuclear membranes and prominent nucleoli.
Figure 4b: On immunohistochemistry, the tumour is glypican-3 positive.