24. Biliary/Liver 2 Flashcards
Signs of pancreatic cancer?
Painless jaundice and a palpable gallbladder - 75% in head of pancreas from exocrine tissue
Rest are MEN1 arising from endocrine tissue
FLAWS Exocrine - steatorrhoea Endocrine - diabetes Hepatomegaly Trossaeu sign (migratory thrombophelbitis)
Investigations for pancreatic cancer?
Bloods - CA19-9
USS
High res CT if number 1 differential - see double duct sign
Gold standard is biopsy by ERCP or US
Management of pancreatic cancer?
ERCP for stenting
Whipple’s resection with adjuvant chemo – side effect would be dumping syndrome (rapid gastric emptying) or PUD
What is a cholangiocarcinoma?
Cancer arising from bile ducts either intrahepatic or extrahepatic.
Signs and symptoms of a cholangiocarcinoma?
Virchow's node Sister mary joseph nodule FLAWS RUQ pain Bile obstruction - jaundice, palpable GB, pruritus, pale stool and dark urine
Investigations for cholangiocarcinoma?
Bloods - LFTs (obstructive), clotting, CA19-9 and CEA
Imaging - USS and MRCP
Radiography - PTC
ERCP with biopsy is gold standard
Management for cholangiocarcinoma?
Depends on where cancer is - Removal of bile duct - Partial hepatectomy - Whipple's proceduce (distal BD tumours) Adjuvant chemo or radiotherapy
What is a hepatocellular carcinoma?
Primary malignancy of hepatocytes
Signs and symptoms of HCC?
Presents late (like rest) FLAWS Chronic liver failure symp - Hepatosplenomegaly Pruritus Jaundice Decompensation - Acute deterioration of liver failure Hepatic encephalopathy
How to investigate HCC?
Screening for RFs e.g. haemochromatosis, alc hep etc.
Bloods - aFP tumour marker
Imaging - USS
How to manage HCC?
Early - Surgical resection (non-cirrhotic) - Transplant (cirrhotic) Unsuitable - Percutaneous ethanol injections - Multikinase inhibitor with sorafenib
What is primary biliary cirrhosis?
Autoimmune condition associated with granuloma formation in the bile ducts. Inflammatory disorder causing destruction of interlobular bile ducts.
More common in women.
Signs and symptoms of PBC?
Pruiritus and jaundice (obstruction) RUQ pain Xanthelasma and xanthomata Clubbing Hepatosplenomegaly
Investigations for PBC?
Bloods - AMA M2 antibodies (raised serum IgM too)
How to treat PBC?
Treat symptoms
Vitamin A, D, E and K supplements
Ursodeoxycholic acid
Cholestyramine for pruritis
Transplant
What is primary sclerosing cholangitis?
Biliary inflammation of unknown aetilogy, inflammation of intra or extrahepatic ducts
Signs and symptoms of PSC?
Pruritus, jaundice, fatigue, RUQ pain
Investigations for PSC?
Bloods - pANCA positive
ERCP/MRCP - bead on a string appearance
Biopsy - onion skin fibrosis
How to manage PSC?
Screen for cholangiorcinoma
Follow up LFTs and tumour markers every 6 months
Proceed to ERCP and biopsies of any suspicious lesions
What is wilson’s disorder?
An autosomal recessive disorder where there is excessive copper deposition in tissues.
Increased copper absorption from SI and decreased hepatic excretion.
Signs and symptoms of wilson’s?
Neuro - parkinsonism, behavioural changes, psychiatric or speech distubances
Eyes - kayser-fleischer rings
Liver - hepatitis and cirrhosis
Investigations wilson’s
Slit lamp eye examination
Bloods LFTs, reduced serum caeruloplasmin and raised free copper
Urinalysis - increased 24hr copper excretion
Genetic testing/liver biopsy
How to manage wilson’s?
Copper chelation with penicillamine
Other chelation - trientine hydrochloride, tertrathiomolybdate
What is haemochromatosis?
Abnormal iron deposition in certain organs causing oxidative damage
Primary - due to mutation on HFE gene, autosomal recessive
Secondary to frequent blood transfusions, iron supplementation or diseases of erythropoiesis
