Hepatobiliary Tumors: Update on Diagnosis and Management

Abstract

Tumors of the liver and biliary tree, mainly hepatocellular carcinoma and cholangiocarcinoma, are the second leading cause of cancer related death worldwide and the sixth leading cause of cancer related death among men in developed countries. Recent developments in biomarkers and imaging modalities have enhanced early detection and accurate diagnosis of these highly fatal malignancies. These advances include serological testing, micro-ribonucleic acids, fluorescence in situ hybridization, contrast-enhanced ultrasound, and hepatobiliary-phase magnetic resonance imaging. In addition, there have been major developments in the surgical and nonsurgical management of these tumors, including expansion of the liver transplantation criteria, new locoregional treatments, and molecularly targeted therapies. In this article, we review various types of hepatobiliary tumors and discuss new developments in their diagnosis and management.

Keywords: Cholangiocarcinoma; Diagnosis; Hepatobiliary tumors; Hepatocellular carcinoma; Liver cancer; Management; Treatment.

Fig. 1. List of malignant and benign hepatobiliary tumors.

Fig. 2. Radiologic images of hepatocellular carcinoma (HCC).

MRI images of HCC showing A, arterial enhancement; B, washout on portal venous phase with delayed capsular enhancement; C, Precontrast MRI showing hypointense lesion in another patient with HCC; D, MRI with 20 min delayed phase Eovist showing diffuse hypointense biopsy-proven HCC.

Fig. 3. Histopathology of malignant intrahepatic lesions.

A, Hepatocellular carcinoma with moderate to marked cytologic atypia. The tumor is highly cellular, without intervening stroma [H&E, ×100]; B, Intrahepatic cholangiocarcinoma showing poorly formed neoplastic glands embedded within sclerotic stroma [H&E, × 200]; C, Fibrolamellar type hepatocellular carcinoma showing abundant eosinophilic granular cytoplasm and prominent nucleoli within an area of dense sclerotic stroma [H&E, ×100]; D, Epitheliod hemangioendothelioma demonstrating hyalinized stroma harboring entrapped blister cells with cytoplasmic lumen. Although the cells appear epithelioid, it is of endothelial origin [H&E, ×200].

Fig. 4. Hepatic hemangioma.

A, Medium magnification showing vascular channels lined by bland and attenuated endothelial cells [H&E, ×100]; B, T2-weighted MRI image showing hyperintense lesion in the left lobe; C, MRI arterial phase showing hypointense well-circumscribed lesion with minimal peripheral arterial enhancement; D, MRI portal venous phase showing avidly enhancing well circumscribed lobulated lesion, which has “filled in” on more delayed phase imaging.

Fig. 5. Pathology and radiology of focal nodular hyperplasia (FNH).

A, Scanning view of FNH demonstrating central stellate shape scar (black arrow) [H&E, ×6.6]; B, FNH showing broad bands of fibrous septa with dystrophic vessels. There is florid bile ductular proliferation along the fibrous septa [H&E, ×100]; MRI of FNH showing C, hypointense lesion on precontrast image; D, rapid arterial enhancement; E, rapid washout to near background on portal venous; F, equilibrium phase with delayed enhancement of central scar.

Fig. 6. Pathology and radiology of hepatocellular adenoma (HCA).

A, HCA showing scattered arteries unaccompanied by portal veins or bile ducts (unpaired arteries) [H&E, ×100]; MRI images showing B, arterial enhancement; C, some washout on portal venous phase; D, hypointensity to liver on hepatobiliary phase.

Fig. 7. Metastatic melanoma.

A, Pathology slide showing frequent pigments admixed within the tumor and prominent cherry-red nucleoli [H&E, ×200]; B, T1-weighted MRI showing multiple hyperintense lesions consistent with melanin or hemorrhage.

Fig. 8. Radiologic imaging of biliary cystadenoma (A, B), and cholangiocarcinoma (C, D).

CT scan showing (A) a heterogenous, complex cystic mass with evidence for (B) biliary dilatation in left hepatic lobe representing biliary cystadenoma. Cholangiocarcinoma appearing as (C) a hypointense mass on T1-weighted MRI and as (D) a hyperintense ill-defined mass on T2-weighted MRI.

Fig. 9. Biliary hamartomas.

A, Hyperintense foci along the biliary tree on T2-weighted images; B, Well-defined hypointense lesions on T1-weighted image postcontrast.