3/29 Masses of Liver and Biliary Tree - Corbett

Question 1

what percentage of total blood flow is received by liver? what fraction portal circ?

significance of hepatic artery blood supply?

Answer 1

28% of total, of which 75% comes from portal vein

hepatic a is sole blood supply of bile ducts

Question 2

formation of bile ducts

Answer 2

from ductal plates

Question 3

choledochal cysts

Answer 3

child presenting with

• CHOLESTASIS
• RUQ pain
• fever
• jaundice
• palpable mass

Question 4

biliary atresia

Answer 4

fibrotic obliteration of extrahepatic biliary system

ex. newborn with persistent jaundice

90% of time = ISOLATED finding

10% splenic malformation

clinical pres

• jaundice is first sign (maybe only in sclerae) - onset b/w birth-8wk
• acholic stools
• dark urine

if onset past 6months? something else!

Question 5

Caroli’s disease

Answer 5

saccular dilatation of intrahep bile ducts

assoc: AR polycystic kidney disease

Question 6

benign lesions of liver

Answer 6

generally asymptomatic

can produce RUQ pain w growth

Question 7

hepatic hemangiomas

Answer 7

• common, often incidental finding
• large lesions (> 4CM) in young women
• ASYMPTOMATIC

features

• tend to be solitary, at periphery
• discrete red-blue soft nodules
• vascular channels in a bed of fibrous connective tissue
• most DONT grow

management

• percutaneous biopsy not recommended → risk of hemorrhage for large lesions
• one concern - incr in size with preg or OCP use
• small lesion (under 4-5cm) → no further studies
• large lesion → resection

Question 8

focal nodular hyperplasia

Answer 8

second most common

65-80% asymptomatic

women (8:1), age 30-40

pathogenesis

• nodular regen
• hyperplastic response to hyperperf by anomalous artery
• role of OCPs uncertain…might promote growth

features

• solitary, < 5cm
• well demarcated but NO CAPSULE
• central stellate scar - abnormal vessels, bile ductule prolif

generally stable over time

90% dx made with imaging: helical CT scan, MRI, contras-enhanced US

Question 9

!! hepatic adenoma !!

Answer 9

uncommon benign epithelial tumor in otherwise normal liver

young women w OCP use

risk factors

• OCP (30-40x) → more numerous, larger, more likely to bleed
• anabolic steroids
• glycogen storage disease (type 1, type 3)
• preg

features

• solitary in 70-80%
• often right lobe
• often 5-15cm at dx

pathology

• large plates of adenoma cells
  
  • larger than normal hepatocytes, contain lipids/glycogen
• lack normal architecture (no bile ductules)
• cells nonfxal

assoc w complications

Question 10

hepatic adenoma

clinical fts

tx

Answer 10

• incidental discovery of a mass on hep imaging
• palpable liver mass
• RUQ abdominal pain

complications

• malignant transformation
  
  • seen in 8-15%
  • rise in AFP
  • incr size
• hemorrhage (can be life threatening)
  
  • larger = more risk

tx: RESECTION

Question 11

hepatic cysts

Answer 11

most are incidental and benign

simple: < 4cm

do not associate w biliary tree

assoc: auto dom PDK → cysts appear as disease progresses

Question 12

ameobic liver abscess

Answer 12

extraintestinal manifestation of Entamoeba histolytica

acute presentation (<14d) w fever, dull aching RUQ

Question 13

pyogenic hepatic abscess

Answer 13

uncommon but fatal if untreated (100% mort if unrecog’d, untx’d)

etiology

• biliary tract
• portal v (appendicitis, pylephlebitis, IBD)
• hepatic a (IVDA)
• cryptogenic (DM, colon cancer)

fever, RUQ pain, tender/enlarged liver, jaundice w/ compressive effect

tx: IV antibiotics

Question 14

invasive liver abscess syndrome

Answer 14

Klebsiella pneumoniae liver abscess is the sole presenting manifestation

• specific to K1 and K2 serotype
• first described in SE Asia

risk: diabetes

clinical: fever, rigors, abd pain

Question 15

hepatoblastoma

Answer 15

most common liver tumor of early childhood

assoc w WNT signaling

APC gene mutation → occurs in FAP families

Question 16

hepatocellular carcinoma

Answer 16

85% of HCC occurs in areas with high chronic HBV inf

vertical transmission of HBV - carrier state starts in infancy

peak: 20s-40s

*50% occurs in absence of cirrhosis*

in USA…

occurs in context of HCV infection

*90% occurs in setting of established cirrhosis

Question 17

HCC

pathogenesis

Answer 17

INFLAMMATION

associated with chronic liver infl

risk factors:

• viral infections (HBV, HCV) - endemic areas
• toxic injuries (aflatoxin, alcohol)
• metabolic disease of liver
  
  • hemochromatosis, alpha1AT def, Wilsons
  • NASH

beta catenin: mediate cell/cell contact

p53: guardian of the genome

Question 18

Budd Chiari example

Answer 18

Question 19

HCC - fibrolamellar type

Answer 19

less than 5% of HCCs

85% occuring below 35yo

no gender more affected, no identifiable predisposing conds

• single, hard, “scirrhous” tumor
• well-diff cells rich in mitochondria

Question 20

metastases TO the liver

Answer 20

metastatic disease is far more common than primary liver tumors

(most common site after lymph nodes!)

common origins:

• colon/rectum
• pancreas, stomach, esophagus, breast
• lung
• melanoma

Question 21

cholangiocarcinoma

Answer 21

malignant tumor of biliary tree

• located within and outside liver

perihilar tumors are most common - aka Klatskin tumor (50% located here)

risk factors

• assoc with chronic infl in bile ducts
  
  • liver fluke infestation (Opisthorchis, Clonorchis)
• primary sclerosing cholangitis
• chronic choledocholithiasis
• HBV, HCV
• NASH

Question 22

angiosarcoma of liver

Answer 22

associated with vinyl chloride and arsenic