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GI > Liver Path: Cholestatic/Neoplastic Disease > Flashcards

Liver Path: Cholestatic/Neoplastic Disease Flashcards

1
Q

What are causes of steatosis? (4)

A

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

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2
Q

Describe the histology of alcoholic liver disease (2)

A

Steatosis with pericentral sinusoidal (arachnoidal/chicken wire) fibrosis

Mallory denk bodies (balloon appearance with eosinophilic hyaline bodies)

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3
Q

NAFLD: describe the prevalence and histology

A

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

Histological features in adults overlap with alcoholic liver disease

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4
Q

Describe the difference between hemochromatosis and hemosiderosis

A

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

Hemosiderosis is secondary hemochromatosis due to other causes

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5
Q

Describe the causes of hemosiderosis (5)

A

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

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6
Q

Describe histology for hereditary hemochromatosis

A

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

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

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7
Q

Describe histology of hemosiderosis

A

Iron accumulation mainly occurs in kuppfer cells

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8
Q

What is cause of neonatal hemochromatosis?

A

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

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9
Q

Describe the intrahepatic causes of cholestasis (5)

A

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

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10
Q

Describe extra hepatic causes of cholestasis (4)

A

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

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11
Q

Compare and contrast primary biliary cirrhosis vs primary sclerosing cholangitis:

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

A

Do it do it

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12
Q

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

A 
Age: median=50yo
Gender: 90% female
Associated conditions: Sjogren, thyroid disease
Serology: AMA, ANA, ANCA
Radiology: normal 
Duct lesion: Inflammatory destruction
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13
Q

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

A 
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
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14
Q

Describe pathophysiology of primary biliary cirrhosis

A

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

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15
Q

Describe histology of PSC

A

Fibrous obliterative ductal lesions

Onion skinning fibrosis around destroyed bile duct

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16
Q

What are causes of ductopenia in adults? (3)

A

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

17
Q

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

A

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

18
Q

What is alagille syndrome?

A

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

19
Q

What are causes of obstructive cholestasis? (4)

A

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

20
Q

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

A

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

21
Q

What histological finding occurs in ascending cholangitis due to gallstones?

A

Increased neutrophils

22
Q

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

A

Biloma: a bile filled cyst in liver

Biliary cirrhosis: due to extravasation of bile within liver

23
Q

Hepatic Infarction: histology

A

Coagulative necrosis of hepatocytes with hyperemic rim on edge of necrosis

Can also observe infarct of major ducts of biliary tree

24
Q

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

A

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

Intrahepatic: schistosomiasis, obliterative portal venopathy

25
Q

What is the cause of Budd Chiari Syndrome?

What are the symptoms? (3)

A

Outflow obstruction due to hepatic vein thrombosis

Symptoms of Budd Chiari include hepatomegaly, ascites and liver dysfunction

26
Q

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

A

Endothelial injury to sinusoids and terminal hepatic venules

Observed in patients post stem cell tx and receiving chemo

27
Q

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

A

Centrilobular congestion
Hepatocellular necrosis
Obliteration of small hepatic veins

28
Q

How does liver appear in CHF? (3)

A

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

29
Q

What is the cause of echninococcal/hyatid cysts?

What is concern during removal?

A

Larval stages of tapeworm

Surgical removal risks rupture which can result in fever, anaphylaxis

30
Q

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

A

Simple cysts– bile duct cyst

Polycystic liver disease

31
Q

What are the two types of vascular tumors in liver?

How do they present?

A

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

Infantile hemangioendothelioma==>most common liver tumor in children

32
Q

What is hepatocellular adenoma associated with? (2)

A 
OCP
Metabolic diseases (i.e glycogen storage disease)
33
Q

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

A

Most frequent solid lesion

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

34
Q

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

A

HCC
Cholagniocarcinoma
Hepatoblastoma

35
Q

Describe the epidemiology of HCC: prevalence, geography, RFs

A

HCC=5% all cancers
Highest incidence in asian countries with high HBV prevalence

RFs: HBV, HCV, Aflatoxin, alcohol, metabolic diseases…anything that causes cirrhosis

36
Q

What are the prognostic factors for HCC? (4)

What is the prognosis for HCC?

A

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

Prognosis is terrible (

37
Q

What is precursor for HCC?

A

Dysplastic lesions

38
Q

What is fibrolamellar carcinoma?

A

A variant of HCC (makes up 5%)

Typically appears

39
Q

Hepatoblastoma: how does it present? what is prognosis?

A

Presents with asymptomatic abdominal mass in children

Better prognosis than HCC

GI (41 decks)

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