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Tumours of the Liver, Gall bladder and Bile duct

 

1. BENIGN LIVER LESIONS

  • Benign liver lesions are more common than malignant tumours

Cyst

  • Most common liver lesion
  • Congenital Cysts
    • More common in females
    • Thin walled, homogenous, fluid-filled structure
    • Cyst is lined by cuboidal epithelium – secretes a clear fluid
    • Usually asymptomatic, unless they are very large
  • Acquired cysts – secondary to trauma, infection or neoplasm

Hemangioma

  • Consist of large endothelial-lined vascular spaces
    • Contain fibrous tissue and small blood vessels that eventually grow
  • They are congenital vascular lesions
  • Can be small (<1cm) or giant (10-25cm)
  • Most discovered incidentally
  • Large lesions may compress adjacent structure and cause symptoms
  • Complications – bleeding, thrombosis, DIC, infection
  • Investigations – CT, MRI
  • Treatment – enucleation/hepatic resection/radiation in adults

Adenoma

  • Benign, solid neoplasms
  • Can be single or multiple (>10 is called adenomatosis)
  • Most common in premenopausal women over 30 years old – use of OCP is a risk factor
  • Histology
    • Lack bile duct glands and Kuppfer cells
    • Don’t have true lobules
    • Hepatocytes are vacuolated due to glycogen deposition
  • Investigations
    • CT – shows sharp borders
    • MRI – with gadoxetate (liver specific MRI contrast)
  • Complications
    • Risk of spontaneous rupture with intraperitoneal bleeding
    • Malignant transformation into hepatocellular carcinoma
  • Treatment – hepatic resection

Focal nodular hyperplasia

  • Benign lesion of liver thought to be a hyperplastic response to an anomalous artery
  • Most common in premenopausal women
  • Histology – contains Kuppfer cells and hepatocytes
  • Investigations
    • CT – shows well circumscribed lesion with central scar
  • Unlike adenomas, they do not rupture spontaneously and have little risk of malignant transformation
  • Treatment – observation and periodic checks

2. HEPATOCELLULAR CARCINOMA

Epidemiology

  • Most common primary malignant neoplasm of liver
  • Most prevalent in areas with high incidence of HBV
  • Common in Africa and China
  • Most common in men 50-60 years old

Etiology

  • HBV, HCV, cirrhosis, non-alcoholic fatty liver disease
  • Aflatoxin (product of fungus aspergillus)
  • Hepatic adenoma

Pathology

  • 3 distinct gross patterns
    • Hanging type – connects to normal liver via vascular stalk (very resectable)
    • Pushing – well demarcated with fibrous capsule. Displaces normal vasculature (resectable)
    • Infiltrative type – invades vascular structures (difficult to resect)
  • Begins as a single tumour but can lead to multiple lesions due to invasion and metastasis

Clinical features

  • Non-specific symptoms – fatigue, fever, weight loss, lethargy
  • Right upper quadrant pain – uncommon, but suggestive of HCC in a cirrhotic patient
  • Enlarged, tender liver
  • Jaundice
  • Ascites

Investigations

  • AFP >500ng/ml (>100 IU)
  • LFTs
  • US abdomen
  • CT – shows size, extent, location, vascularity, portal vein invasion and nodal status
  • CT angiography
  • Biopsy –definitive

Staging

Barcelona Clinic Liver Cancer (BCLC) Classification

Treatment

  • Curative – partial hepatectomy, liver transplant
  • Palliative – embolisation, thermal/radiofrequency ablation
    • Chemo has limited benefits

3. GALLBLADDER CANCER

  • Aggressive malignant disease – poor prognosis
  • Usually presents at a late stage

Epidemiology

  • Most common in 60-70 year olds, especially in females
  • Highest incidence in India and Pakistan

Etiology

  • >90% of patients have associated Cholelithiasis
  • Risk factors – obesity, female sex, primary sclerosing cholangitis

Pathology

  • Adenocarcinoma is the most common type
  • Other types – squamous cell carcinoma, adenosquamous carcinoma, carcinoid tumour
  • Gradual progression from dysplasia → carcinoma in situ → invasive carcinoma
  • Mainly found in fundus/body of the gallbladder

Spread

  • Direct spread – to liver (segment IV and V), bile duct, duodenum, colon kidney
  • Lymphatic – lymph node of Lund, peripancreatic and periduodenal nodes
  • Haematogenous – liver, lungs, bones

Clinical features

  • Most are asymptomatic until advanced disease
  • Weight loss, jaundice, abdominal mass
  • Chronic epigastric pain
  • Early satiety
  • Courvoisier’s law – presence of a palpably enlarged, non-tender gallbladder accompanied by mild jaundice is unlikely to be gallstones and is presumed to be due to malignancy of the gallbladder or pancreas

Investigations

  • US – shows irregular shaped and asymmetrical thickened gallbladder wall
  • CT for staging
  • MRCP
  • CA 19-9

Treatment

  • Surgery – only curative option
  • Cholecystectomy with resection of liver segment IV + V
    • Clear all pericholedochal lymph nodes
  • Palliative procedure – endoscopic/percutaneous stents to treat jaundice

4. CHOLANGIOCARCINOMA

  • Rare tumour arising from biliary epithelium – can occur anywhere in biliary tree
  • Most commonly localised at hepatic duct bifurcation
  • Usually presents when disease is advanced
  • Age of presentation – >50 years old

Etiology

  • Associated with
    • Caroli’s disease – inherited, cystic dilation of intrahepatic bile ducts
    • Clonorchiasis – Chinese liver fluke parasite
  • Risk factor – primary sclerosing cholangitis, choledocal cyst, HBV/HCV

Pathology

  • 95% are adenocarcinomas
  • Locations
    • Intrahepatic
    • Perihilar/bifurcation – AKA Klatskin tumours (further classified in the Bismuth classification – see below)
    • Distal

Classification – Bismuth Classification

  • I – limited to the common hepatic duct, below the confluence of the right and left hepatic ducts
  • II – involves the confluence of the right and left hepatic duct
  • III – (a) type II and extends into right hepatic duct (b) type II and extends into the left hepatic duct
  • IV – extends to the bifurcations of both right and left hepatic ducts

Clinical features

  • Klatskin tumour is more likely to present with obstructive jaundice
  • Intrahepatic tumour is more likely to present with a liver mass
  • Painless jaundice, pruritis
  • Right upper quadrant pain
  • Weight loss, anorexia, fatigue

Investigations

  • Blood – ↑ALP, ↑GGT,↑CA 19-9
  • MRCP/ERCP
  • US, CT

Treatment

  • Surgical excision – only curative treatment
  • For intrahepatic or Klatskin tumours
    • Partial hepatectomy, cholecystectomy
  • For distal bile duct tumours – Whipple’s (pancreaticoduodenectomy)
    • Pancreaticojejunostomy, gastrojejunostomy, choledojejunostomy
  • Palliative treatment
    • Roux-en-Y hepaticojejunostomy
    • Insertion of stents
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