neoplasms of biliary tract & liver Flashcards
what is cholestasis
- systemic retention of bilirubin/ other solutes eliminated in bile, caused by impaired bile formation and bile flow
presentation of cholestasis
jaundice
- yellow discolouration of skin
icterus
- yellow discolouration of sclera
other symptoms
- pruritus, skin xanthomas, intestinal malabsorption / vit ADEK deficiencies
types of jaundice
Pre-hepatic:
* excess production of bilirubin e.g. haemolysis, ineffective erythropoiesis
Hepatic:
* reduced hepatic uptake e.g. drugs
* impaired bilirubin conjugation e.g. physiologic / neonatal jaundice, genetic deficiency, diffuse hepatocellular disease
* impaired bile flow e.g. AI cholangiopathies
Post-hepatic:
* impaired bile flow / large duct obstruction
what does post hepatic jaundice commonly present with
- hyperbilirubinaemia
unconjugated vs conjugated hyperbilirubinaemia
unconjugated
- before conjugation, pre hepatic
- excess cannot be excreted in urine
conjugated
- after conjugation in liver
- water soluble -> excess CAN be excreted in urine
Biliary tract diseases
- large bile duct obstruction
- primary heptolithiasis
- neonatal cholestasis & biliary atresia
- structural anomalies of biliary tree
what is large bile duct obstruction associated with (2)
- GALLSTONES
- MALIGNANCIES of biliary tree
others:
- inflammation of bile duct strictures
- porta hepatis lymphadenopathy
- bile duct malformations in children
large bile duct obstruction symptoms
- acute: reversible with correction of obstruction
- subtotal/ intermittent obstruction: increase risk of ascending cholangitis -> abscess & sepsis
- chronic: biliary cirrhosis
what is primary hepatolithiasis
- intrahepatic biliary stone formation
- lead to repeated bouts of ascending cholangitis, progressive inflammatory destruction -> collapse & scarring of hepatic parenchyma
what does primary hepatolithiasis predispose pt to
- Biliary Intraepithelial Neoplasia (BilIN) and cholangiocarcinoma
neonatal cholestasis
- prolonged conjugated hyperbilirubinaemia in neonate BEYOND 14 DAYS after birth
most common cause of neonatal cholestasis
- cholangiopathies (extrahepatic biliary atresia)
what is extrahepatic biliary atresia
- complete/ partial obstruction of lumen of extrahepatic biliary tree within FIRST 3 MONTHS of life -> can extend to involve intrahepatic ducts
types of structural anomaly of biliary tree
- choledochal cyst
- fibropolycystic disease
choledochal cyst
- developmental malformation of biliary tree, usually CBD -> predispose to stones, stenosis, strictures, pancreatitis and risk of bile duct cancer
fibropolycystic disease
- Heterogeneous group of lesions in which the primary abnormalities are congenital malformations of the biliary tree (ductal plate malformations)
diseases of gallbladder
- cholelithiasis
- cholecystitis (acute & chronic)
clinical features of cholelithiasis
- usually asymptomatic
- RUQ/ epigastric pain ->
complications of gallstones
- obstruction, pancreatitis
- gallbladder carcinoma
- perforation
acute cholecystitis (gall bladder inflammation) presentation
- progressive pain >6 hrs
- fever, sweating, N&V
- NO JAUNDICE (if present -> suggest CBD obstruction)
calculous acute cholecystitis (90%)
- obstruction of neck/ cystic duct by GALLSTONES -> chemical irritation & inflammation
- primary complication of gallstones
acalculous acute cholecystitis (10%)
- found in severely ill pts (sepsis, DM, infection, immunosuppressed)
- due to ISCHAEMIA (cystic artery is end artery)
- presence of inflammation & edema of wall -> further compromise blood flow -> gallbladder stasis, biliary sludge & gallbladder mucus cause obstruction WITHOUT stones (acalculous)
chronic cholecystitis
- contracted thickened wall due to chronic inflammatory infiltrates
- fibromuscular hypertrophy
types of non neoplastic mass
- focal nodular hyperplasia
focal nodular hyperplasia
- due to focal alterations in hepatic blood supply
most common benign liver tumour
- cavernous haemangioma
- hepatocellular adenoma
gross feature cavernous haemangioma
- subcapsular, soft, red blue
complications: rupture -> intraperitoneal bleeding
hepatocellular adenoma presentation
- benign tumour arising from hepatocytes
- incidental, abdo pain from rapid growth or haemorrhage, intraabdominal bleeding due to rupture (surgical emergency)
hepatocellular adenoma risk factor
- oral contraceptives
- anabolic steroids
rank most commonly found malignant tumour of liver
- # 1 - hepatocellular carcinoma (HCC)
- # 2 - cholangiocarcinoma
hepatocellular carcinoma etiology
- countries with high rates of CHRONIC HBV (hep B) infection -> china, kr, taiwan
- increasing in west due to Hep C epidemic
**major cause - viral infection (HBV, HCV)
pathogenesis of hepatocellular carcinoma
- beta-catenin activation -> demonstrate genetic instability, unrelated to HBV
- p53 inactivation
features of hepatocellular carcinoma
- hepatomegaly
- upper abdo pain, fatigue
cholangiocarcinoma pathogenesis
- carcinoma of bile duct origin
- sporadic; usually chronic inflammation & cholestasis -> promote somatic mutation & epigenetic alterations
*POOR PROGNOSIS
cholangiocarcinoma presentations
affected by location
- extrahepatic -> present earlier & smaller with biliary obstruction, cholangitis, RUQ pain
- intrahepatic tumours -> usually undetected till late
most common liver tumour of EARLY CHILDHOOD (<3yo)
hepatoblastoma
what is hepatoblastoma associated with
- FAP
- Beckwith Wiedemann syndrome
are primary hepatic neoplasms are secondary hepatic neoplasms more common
- SECONDARY METASTASES»_space;> primary
common primary sites for secondary hepatic metastases
- colon, breast, lung, pancreas
secondary hepatic metastases presentations
- usually asymptomatic, liver function retained
gross imaging of secondary hepatic metastases
- hepatomegaly
- multiple pale nodules in non-cirrhotic liver
- central tumour necrosis causing subcapsular umbilication of nodules
most common malignancy of EXTRAHEPATIC BILIARY TRACT
- gallbladder carcinoma
which gender is gallbladder carcinoma more common in
female (2:1 F:M)
risk factors of gallbladder carcinoma
- gallstones (95%); though little people (1%) with gallstones develop the carcinoma
- chronic bacterial & parasitic infection
how can gallbladder carcinoma metastases
direct invasion
- liver, stomach, duodenum
metastases
- liver, regional lymph nodes, lungs
**POOR PROGNOSIS
