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 Diagnosis
 Fibro lamellar variant of HCC versus Fibronodular hyperplasia

Case 2

                                       Contributors

                                                 Dr.Ambika Sunil Gayad                                                           

 Radiological Findings, Disease course, and Management

 

 Ultrasound examination of liver

 

 

 

 

 

 

 

 

 

   

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Findings

Two lesions seen in the liver

larger one appears mixed echoic, predominantly hyperechoic ith large central scar with coarse calcifications. No significant internal vascularity

 

 CT

 

               

                                                  

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Findings

  • Liver measures 14 cm, shows irregular lobulated margins with surface irregularities

  • volume redistribution in the form of left lobe and caudate lobe hypertrophy with relative atrophy of right lobe.

  • evidence of an irregular, fairly well-circumscribed, lobulated mass lesion approximately measuring 78 x 60  x 64 mm ( TR x AP x CC) in the right lobe, involving segments V / VI, demonstrating stellate shaped central scar with calcifications.

  • Adjacent liver shows capsular retraction.

  • Post contrast study: the lesion demonstrates enhancement in the arterial phase with washout in the venous and delayed phase images.

  • No significant enhancement of the central scar on delayed phase images.

  • Another ill-defined hypodense lesion is seen approximately measuring 13x15 mm in the subcapsular location of segment VII of liver.

  •  Main portal vein is measures 15 mm, shows normal contrast opacification.No regional lymphadenopathy.Intra-hepatic biliary radicals are not dilated.

  • Mild degree free fluid is seen in the pelvis, peri-hepatic space and right paracolic gutter.

  • CBD is normal in calibre ( measures 4 mm).Gall Bladder is minimally distended and shows multiple tiny calculi, largest measuring 6 mm.

  • No evidence of pericholecystic collection / wall thickening.

  • Spleen is normal in size (10.5 cm) , shape, density and enhancement pattern. No focal lesion seen.

  • Pancreas shows diffuse pancreatic parenchymal atrophy with multiple intra-parenchymal calcifications. Pancreas shows normal density and enhancement pattern. No focal lesion. Main pancreatic duct is mildly prominent, measuring 3 mm in the tail region.No obvious intraductal calculi. No evidence of peripancreatic collection / pseudocyst formatio

Impression

  • Chronic liver parenchymal disease / cirrhosis with a space occupying lesion in the right lobe of liver with CT morphology as described above.

 

 

    Possible differentials:

           1. Fibrolamellar variant of hepatocellular carcinoma……More likely

           2. Focal nodular hyperplasia, Less likely​

  • Another small hypodense lesicapsular location of segment VII of liver

  • Cholelithiasis

  • Chronic calcific pancreatitis

  • Mild ascites

 

On Follow up: Serum AFP - normal

 

Discussion

   Background

  • very rare hepatic tumor (approximately 1% of all hepatic tumors and less than 10% of all hepatocellular carcinomas)

  • mainly affecting young healthy female patients without underlying liver disease 

  • better prognosis than the usual HCC 

  • absence of relevant symptoms may lead to their large size and/or extra-hepatic disease at initial presentation

  • Metastatic lymphadenopathy is the most frequent extra-hepatic finding

  • Alpha-FP levels:typically normal

 Diagnosis

 Imaging findings

 most important criteria for its diagnosis are

  • detection of coarse central calcifications (15-25% of cases) and hypervascular features on dynamic study

  • demonstration of a hypointense central scar on MRI, both on T1- and T2-w images, also showing lack of enhancement.

Differential diagnosis

include conditions such as focal nodular hyperplasia.

  • this benign tumor exceptionally contains calcifications and generally displays a T2-w hyperintense and vascularized central scar, in contrast to FL-HCC 

  • On pathology the characteristic pattern of collagenous lamellar fibrosis, relatively acellular, is the histological hallmark of FL-HCC

Treatment and prognosis

  • Surgical resection or transplantation is the standard of care for fibrolamellar carcinoma (FLC) for eligible patients 

  • Trans-arterial chemo-embolization (TACE) may be a useful option in patients who have unresectable disease

  • Chemotherapy for metastatic disease is as for metastatic typical hepatocellular carcinoma (HCC). 

  Conclusion

  • Patients with FLC have many clinicopathologic features that are different from those of patients with HCC, including younger age and female sex.

  • Despite a higher likelihood of advanced disease at the time of diagnosis, surgically treated FLC patients had better long-term outcomes than patients with conventional HCC.

  References

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