Liver Path: Cholestatic/Neoplastic Disease

Question 1

What are causes of steatosis? (4)

Answer 1

Alcoholic liver disease
NAFLD
Ischemia
Inherited disorders: FAO defects, mitochondriopathies, galctosemia, fructose intolerance, CF, cholesterol storage disorder

Question 2

Describe the histology of alcoholic liver disease (2)

Answer 2

Steatosis with pericentral sinusoidal (arachnoidal/chicken wire) fibrosis

Mallory denk bodies (balloon appearance with eosinophilic hyaline bodies)

Question 3

NAFLD: describe the prevalence and histology

Answer 3

Most common cause of chronic liver disease in US (3-5% population)

Histological features in adults overlap with alcoholic liver disease

Question 4

Describe the difference between hemochromatosis and hemosiderosis

Answer 4

Hemochromatosis: due to gene mutation in HFE, transferrin receptor, hepcidin or HJV– it is hereditary

Hemosiderosis is secondary hemochromatosis due to other causes

Question 5

Describe the causes of hemosiderosis (5)

Answer 5

Parenteral iron overload, Thalassemia
Increased oral intake
Chronic liver disease
Neonatal hemochromatosis

Question 6

Describe histology for hereditary hemochromatosis

Answer 6

Using prussian blue stain, can see iron deposition in hepatocytes (it is golden-brown)

Also observe iron deposition in pancreas, heart, joints, endocrine organs

Question 7

Describe histology of hemosiderosis

Answer 7

Iron accumulation mainly occurs in kuppfer cells

Question 8

What is cause of neonatal hemochromatosis?

Answer 8

Prenatal onset: in-utero allimmune reaction of mother with formation of anti-liver antibodies resulting in liver/renal failure

Question 9

Describe the intrahepatic causes of cholestasis (5)

Answer 9

Diffuse hepatocellular disease (sepsis, hepatitis, cirrhosis)
Canalicular membrane changes (drugs/pregnancy)
Genetic defects in cholestatic transporters (Dubin-Johnson, Rotor)
Primary biliary cirrhosis
Ductopenia

Question 10

Describe extra hepatic causes of cholestasis (4)

Answer 10

Bile duct stones
Intra/extra biliary tumors
Sclerosing cholangitis
Biliary atresia

Question 11

Compare and contrast primary biliary cirrhosis vs primary sclerosing cholangitis:

Age, gender, associated conditions, serology, radiology, duct lesion

Answer 11

Do it do it

Question 12

Describe PBC: Age, gender, associated conditions, serology, radiology, duct lesion

Answer 12

Age: median=50yo
Gender: 90% female
Associated conditions: Sjogren, thyroid disease
Serology: AMA, ANA, ANCA
Radiology: normal 
Duct lesion: Inflammatory destruction

Question 13

Describe PSC: Age, gender, associated conditions, serology, radiology, duct lesion

Answer 13

Age: 30 yo
Gender: 70% male
Associated conditions: IBD, AIH
Serology: ANCA
Radiology: strictures and beading of large extra hepatic ducts
Duct lesion: onion skin fibrosis

Question 14

Describe pathophysiology of primary biliary cirrhosis

Answer 14

Inflammatory destruction of interlobular bile ducts with granulomas leading to cirrhosis with ductopenia

Question 15

Describe histology of PSC

Answer 15

Fibrous obliterative ductal lesions

Onion skinning fibrosis around destroyed bile duct

Question 16

What are causes of ductopenia in adults? (3)

Answer 16

Immune: PBC, PSC, sarcoidosis, rejection, GVHD
Secondary: mechanical, drugs
Idiopathic

Question 17

What are causes of ductopenia in pediatric populations? (4)

Answer 17

Syndromatic: Alagille syndrome
Idiopathic
Metabolic: A1AT deficiency
Secondary: biliary atresia

Question 18

What is alagille syndrome?

Answer 18

Autosomal dominant disorder leading to pathology in liver, heart, skeleton, eye, face, kidney, vasculature

Question 19

What are causes of obstructive cholestasis? (4)

Answer 19

Cholelithiasis (gallstones)
Malignancy of biliary tree of pancreas head
Strictures from surgery
Biliary atresia (children)

Question 20

What are histological findings of extra hepatic bile duct obstruction? (4)

Answer 20

Portal fibrous expansion
Bile duct proliferation
Bile plugs
Feathery degeneration of hepatocytes

Question 21

What histological finding occurs in ascending cholangitis due to gallstones?

Answer 21

Increased neutrophils

Question 22

What are pathology findings in late duct obstruction? Histological and gross

Answer 22

Biloma: a bile filled cyst in liver

Biliary cirrhosis: due to extravasation of bile within liver

Question 23

Hepatic Infarction: histology

Answer 23

Coagulative necrosis of hepatocytes with hyperemic rim on edge of necrosis

Can also observe infarct of major ducts of biliary tree

Question 24

Describe causes of portal venous obstruction: both extrahepatic (4) and intrahepatic (2)

Answer 24

Extrahepatic: neonatal umbilical vein catheterization, intraabdominal sepsis, hypercoagulable disorders, pancreatitis

Intrahepatic: schistosomiasis, obliterative portal venopathy

Question 25

What is the cause of Budd Chiari Syndrome? What are the symptoms? (3)

Answer 25

Outflow obstruction due to hepatic vein thrombosis Symptoms of Budd Chiari include hepatomegaly, ascites and liver dysfunction

Question 26

What is sinusoidal obstruction syndrome (aka veno-occlusive disease)?

Answer 26

Endothelial injury to sinusoids and terminal hepatic venules Observed in patients post stem cell tx and receiving chemo

Question 27

What are histological findings in sinusoidal obstruction syndrome? (3)

Answer 27

Centrilobular congestion Hepatocellular necrosis Obliteration of small hepatic veins

Question 28

How does liver appear in CHF? (3)

Answer 28

Congestion features similar to venous obstruction confined to zone 3 Nutmeg liver Later as sinusoidal fibrosis occurs, septa connect hepatic and portal veins, forming cardiac sclerosis/cirrhosis

Question 29

What is the cause of echninococcal/hyatid cysts? What is concern during removal?

Answer 29

Larval stages of tapeworm Surgical removal risks rupture which can result in fever, anaphylaxis

Question 30

What are other types of cysts observed in liver? (2)

Answer 30

Simple cysts-- bile duct cyst | Polycystic liver disease

Question 31

What are the two types of vascular tumors in liver? How do they present?

Answer 31

Carvernous hemangioma==>most common liver tumor in adults; often asymptomatic or produces abd pain Infantile hemangioendothelioma==>most common liver tumor in children

Question 32

What is hepatocellular adenoma associated with? (2)

Answer 32

``` OCP Metabolic diseases (i.e glycogen storage disease) ```

Question 33

Describe focal nodular hyperplasia-- Epidemiology (prevalence, demographic) Gross appearance

Answer 33

Most frequent solid lesion 3% of population, young females Surrounding liver is normal; there is a central stellate scar

Question 34

What are the types of malignant epithelial liver tumors? (3)

Answer 34

HCC Cholagniocarcinoma Hepatoblastoma

Question 35

Describe the epidemiology of HCC: prevalence, geography, RFs

Answer 35

HCC=5% all cancers Highest incidence in asian countries with high HBV prevalence RFs: HBV, HCV, Aflatoxin, alcohol, metabolic diseases...anything that causes cirrhosis

Question 36

What are the prognostic factors for HCC? (4) What is the prognosis for HCC?

Answer 36

Vascular invasion Differentiation Number/size of nodules Extra hepatic spread (IVC, lungs, regional lymph nodes) Prognosis is terrible (

Question 37

What is precursor for HCC?

Answer 37

Dysplastic lesions

Question 38

What is fibrolamellar carcinoma?

Answer 38

A variant of HCC (makes up 5%) | Typically appears

Question 39

Hepatoblastoma: how does it present? what is prognosis?

Answer 39

Presents with asymptomatic abdominal mass in children Better prognosis than HCC