Pathogenesis of Cirrhosis/Liver Tumors

Question 1

dual blood supply to liver

Answer 1

hepatic artery and portal vein

Question 2

is fibrosis reversible?

Answer 2

reversible in early stage, irreversible in late stage

regeneration + fibrosis –> eventual cirrhosis

Question 3

what types of cells are important in leading to collagen deposition and cirrhosis?

Answer 3

stellate cells b/t hepatocyte and sinusoid

Question 4

stages of cirrhosis

Answer 4

1. portal
2. periportan
3. bridging
4. cirrhosis

Question 5

causes of cirrhosis

Answer 5

• chronic viral hepatitis
• NASH
• alcoholic liver disease
• biliary diseases
• metabolic storage diseases:
  
  • A1AT deficiency
  • primary hemochromatosis
  • Wilson’s disease

Question 6

Answer 6

intracellular A1 globules in A1AT deficiency

Question 7

sx of hepatic failure

Answer 7

• jaundice
• hypoalbuminemia –> peripheral edema, ascites
• coagulopathy (2, 7, 9, 10) –> GI bleeds
• hyperammonemia –> encephalopathy –> coma

Question 8

types of primary liver tumors originating from hepatocyte

Answer 8

benign: hepatic adenoma, focal nodular hyperplasia
malignant: HCC, hepatoblastoma

Question 9

types of primary liver tumors resulting from biliary epithelium

Answer 9

benign: bile duct adenoma, biliary hamartoma
malignant: cholangiocarcinoma

Question 10

hepatocellular adenoma

Answer 10

epi: mostly females, 20s-30s

Risk factors: assoc w/ oral contraceptives, androgenic steroids, mutations in HNF1alpha and beta-catenin, MODY3

presentation: RUQ mass, pain, hemorrhage
dx: CT, biopsy
tx: resection if large

Question 11

Answer 11

hepatocellular adenoma

hepatocytes normal, but lackig portal area w/ bile ducts

vessels –> risk of hemorrhage

Question 12

focal nodular hyperplasia

Answer 12

benign tumor of hepatocytes

usually asymptomatic

histology: fibrous septae, central scar, bile ductal proliferation – hard to distinguish from cirrhosis

Question 13

Answer 13

focal nodular hyperplasia

• bile duct proliferation not seen in adenoma
• fibrous bands on trichrome stain

Question 14

hepatocellular carcinoma - epi

Answer 14

malignant tumor of hepatocytes –> lots of deaths

male predominant

80% assoc w/ chronic HBV or HCV

Question 15

Answer 15

cirrhotic liver with nodules. dysplastic nodules can lead to HCC.

Question 16

Answer 16

HCC w/ portal vein invasion

Question 17

fibrolamellar hepatocellular Ca

Answer 17

special type of HCC w/ no association to chronic liver disease

more common in age 20-40

better prognosis

Question 18

cholangiocarcinoma

Answer 18

adenocarcinoma arising from bile duct epithelium

can be intrahepatic, hilar, or extrahepatic

Question 19

etiology of cholangiocarcinoma

Answer 19

• primary sclerosing cholangitis
• liver flukes
• hepatolithiasis
• fibrocystic liver disease

Question 20

hepatoblastoma

Answer 20

occurs mostly <5 yo

enlarging upper abdominal mass

elevated AFT (alpha-fetoprotein)

sx: virilization and precocious puberty b/c excess hCG production

Question 21

Answer 21

intrahepatic cholangiocarcinoma

Question 22

Answer 22

hilar cholangiocarcinoma