Liver Pathology 3 Flashcards

1
Q

Define the HELLP syndrome

A

Stands for Hemolysis, Elevated Liver enzymes, and Low Platelets

Eclampsia with HTN, proteinuria, peripheral edema, and coagulation abnormalities. Liver can develop hemorrhagic ischemic necrosis

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2
Q

Define acute fatty liver of pregnancy

A

THere is a drmatic onset of liver dysfunction

rare cases can result in acute hepatic failure and death

Liver biopsy shows microvesicular steatosis - probably related to defect in mitochondrial fatty acid oxidation

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3
Q

Define intrahepatic cholestasis of pregnancy

A

mild increase in serum conjugated bilirubin

thought to be related to estrogenic hormones with biliary secretory defects

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4
Q

Describe the type of abnormalities that can occur in pateitns receiving a bone marrow transplant?

A

drug toxicity from immunosippressants

sinusoidal obstruction syndromes

acute and chronic graft s host disease - you get portal inflammation with lymphocytic cholanigitis leading to “vanishing bile ducts”

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5
Q

Describe the type of abnormalities that can ocur in patients receiving a liver transplant.

A

preservation injury (ROS damages organ)

anastomotic problems

acute or chronic rejection

acute you’ll have portal hepatitis with lymphocytic cholangitis and endotheliitis
chronic - inflammatory damage to both bile ducts and arteries

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6
Q

What is a hemagionma?

A

discrete red-blue hemorrhagic nodules composed of dilated endothelial lined bloood-filled channels

often incidental finding

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7
Q

What is a simple liver cyst?

A

a single or small cluster of cysts composed to biliary epithelium detached from the biliary tree

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8
Q

What is polycystic liver disease?

A

multiple cysts - usuallyl associated with polycystic kindey disease

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9
Q

What is the most common neoplasm of the liver?

A

hemangioma

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10
Q

WHat is a choledochal cyst?

A

congenitla dilatation of the common bile duct in children

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11
Q

What are the complications of a choledochal cyst?

A

leads to stasis, biliary obstruction, stones or bile duct carcinoma

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12
Q

What is Caroli’s disease?

A

several congenital disorders resulting in intrahepatic biliary dilattations which communicate with the biliary tree

usually suffer bouts of cholangitis. If this is associated with congenital hepatic fibrosis, it’s Caroli’s syndrome

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13
Q

What is hepatic fibrosis?

A

non-cirrhotic fibrotic liver disease in children - NOT true cirrohsis because there’s no nodular regeneration

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14
Q

Define focal nodular hyperplasia.

A

a well-demarcated lesion composed of a proliferaiton of all liver parenchymal elements

probably a hyperplastic response to a localized vacular occlusive event

no known malignant potential

characteristically forms a mass with a central fibrous scar in a stellate configuration

most common in young adult females - usually discovered incidentally

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15
Q

What is nodular regenerative hyperplasia?

A

A diffuse nonfibrosing nodular hyperplasia of the liver which is easier to see grossly but challenign microscpoically

may get portal hypertension but most are asymptomatic

occurs in association with conditions affectin intrahepatic blod flow like vasculitis, or transplant - probalby a compensatory hyperplasia

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16
Q

Describe a characteristic of focal nodular hyperplasia that may be seen on imaging?

A

Angiography shows a distinctive peripheral filling pattern, and the central scar may be seen with imaging studies.

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17
Q

List a key complication of nodular regenerative hyperplasia.

A

portal hypertension

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18
Q

What is a hepatocellulr adenoma?

A

a true neoplasm, but benign

well differentiated hepatocytes but NO portal triads or central veins. Otherwise just look slike normal liver microscopically

can cause massive bleeding to hemorrhagic necrosis

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19
Q

Describe the characteristic population that can get a hepatocellular adenoma.

A

most commonly occuring in young women with prolonged exposure to oral contraceptives or in weight lifters on steroids

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20
Q

What is the gross appearance of a bile duct hamartoma?

A

grossly appears as a single or more commonly multiple small white nodules that mimic metastatic carcinoma

it’s just a diosordered collection of ectatic bile ducts in a fibrous stroma

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21
Q

What is the gross appearance of a bile duct adenoma?

A

usually a solitary lesions consisting oa benign proliferation of bile ducts (as with a hamartoma, can look like a matastatic carcinoma grossly)

so always obtain a microscopic confirmation of lesions before you just call it metastatic carcinoma

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22
Q

Describe the CT imaging findings of malignancies metastatic to the liver>

A

you’ll see multiple nodules ranging in size

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23
Q

How are metastatic tumors diagnosed?

A

Can be idfficult - people can have normal liver function despite tons of mets

diagnose with imaging and biopsy

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24
Q

Describe the risk factors of hepatocelulr carcinoma.

A

chronic liver disease (any of them)

  • chronic viral hepatitis
  • exposure to toxins like aflatoxin

cirrhosis (from hepatitis, alcohol, hemochromatosis, AIAT def, etc)

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25
Q

What is the gross appearance of hepatocellular carincoma?

A

can be either a solitary mass or multiple nodules

can be diffusely infiltrative

typically SOFT AND HEMORRHAGIC

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26
Q

Compare HCC occurring in the US vs China with regard to patient population.

A

Rare in the US - usually in patients over 60 yo

China - high endemic HBV infection, so it occurs at a younger age (20-40)

male predominance in both

27
Q

What procedure can be used to screen for HCC?

A

the clinical manifestations of HCC can be masked by someone’s underlying cirrhosis or hepatitis, so screening may be very beneficial

elevated serum alpha-fetoprotein (really high) can help, but it’s not specific

best way to screen is therefore imaging with high resolution US or CT

note that needle biopsy might be bad because you can seed the tumor along the needle tract

28
Q

Contrast the fibrolamellar variant of HCC with typical HCC.

A

It’s an HCC composed of polygonal oncocytic tumor cells separated by cords of fibrous stroma (where the lamellar comes from)

occcurs in young adults WITHOUT cirrhosis or viral hepatitis

better prognosis!

29
Q

What are the characteristic microscopic appearances of fibrolammelar HCC?

A

this one is firm grossly because of the fibrous tissue (unlike the usually soft HCC)

typical microscopic with fibrous cords and intervening neoplastic hepatocytes

30
Q

What is cholangiocarcinoma?

A

carcinoma arising form intrahepatic or extrahepatic bile ducts- virtually always adenocarcinomas

tumors are white and hard

31
Q

What are some risk factors for intrahepatic cholangiocarcinoma?

A

anything causeing chronic cholangitis - infection wiht liver flukes, PSC, Caroli’s disease, congenital hepatic fibrosis and choledochal cysts

viral hep B and C, and non-fatty liver disease.

usually older individuals, very poor prognosis

32
Q

What entity would need to be excluded before one could make the diagnosis of intrahepatic cholangiocarcinoma?

A

metastatic adenocarcinoma

it’s a diagnosis of exclusion

33
Q

What is hepatoblstoma of the liver?

A

malignancy composed of immature hepatocytic elemtns (epithelial or mixed epithelial-mesenchyma)

super rare - in young kids

34
Q

What is angiosarcoma of the liver?

A

malignancy of endothelial cells with anastomosing vascular channels lined by malignant cells showing endothelial cell differentiation

risk factor = vinyl chlorid

very aggresive, poor prognosis

35
Q

Define the phrygian cap of the gallbladder?

A

when the fundus is folded over on itself

36
Q

Describe risk factors for the formation of cholesterol gallstones.

A
1. increasing age (over 40)
2, Obesity/metabolic syndrome
3. female gender
4. multiparity
5. rapid weight loss
6 .some drugs
7. stasis of the gallbladder

(4 Fs - fat, female fertile and forty)

37
Q

Describe risk factors for the formation of pigment gallstones?

A
  1. hemolysis

2. biliary tract infections

38
Q

What imaging modality is typically used to detect gallstones?

A

only 10-15% will be radiopaque, so the best way to visualize stones is with US

39
Q

List some complications of gallstones.

A
  1. biliary colid
  2. acute cholecystitis
  3. chronic cholecystitis
  4. choledocholithiaisis
  5. ascending cholangitis
40
Q

List some complications of acute cholecystitis.

A

perforation, bile peritonitis, ascending cholangitis, sepsis, fistula, and gallstone ileus

41
Q

What is choledocholithiasis?

A

stones in the common bile duct

42
Q

What is the most common cause of extrahpetic obstruction?

A

choledocholithiasis

43
Q

Are most gallstones symptomatic?

A

no - 70-80% will be asymptomatic

44
Q

Define chronic cholecystitis>

A

almost always associated with cholelithiasis

supersaturated bile may lead to chronic inflammation and formation of gallstones. you get varying degree of chronic lymphocytic inflammation and fibrosis

45
Q

What is a porcelain gallbladder?

A

chronic choleycstitis can cause dystrophic calcificaitn of the gallbladder wall, which causes poercelain gallbladder

46
Q

Describe the gross appearance of cholesterolosis.

A

subepithelial accumulations of lipid-laden macrophages. looks like little yellow mucosal flecks

basically due to accumulation of cholesterol without clinical significance,

47
Q

What is the gross appearance of a cholesterol polyp?

A

aggregated cholesterolosis can form a polyp

48
Q

What is the gross appearance of a mucocele of the gallbladder/

A

total obstruction of the cystic duct or neck of the GB

you get pressure atrophy of the mucosa so that the GB gets turned into a fluid-filled sac, very thin due to the atophy

49
Q

Define and describe adenoma of the gallblader.

A

true neoplasm, benign

papillary appearance - exactly like adenomas everywhre else in the GI tract. almpst always asymptomatic, but can develop invasic adenocarcinoma

50
Q

Define and describe an adenomyoma of the gallbladder.

A

typically located at the fundus

lesions consists of gallbladder diverticula with focal muscular hyertrophy. Not a true neoplasm, but the thickened wall makes surgeons worry. t’=it’s just muscular hyperplasia of the rokitansky aschoff sinuses

51
Q

Which is neoplastic - an adenoma or adenomyoma of the GB?

A

adenoma

52
Q

State a risk factor for gallbladder carcinoma.

A

typically associated with gallstones in older individuals (more frequent in females)

pathogenesis probably related to irritative trauma or chronicinflammation

53
Q

Why is the survival rate for carcinoma of the GB so low/

A

because we don’t discover them until they’re progressed - incidentally when we go in to remove stones. Usually has metastasized already

54
Q

What is the most common type of GB carcinoma?

A

Adenocarcinoma

5% are squamous

55
Q

State ,by location, the three types of cholangiocarcinomas.

A

intrahepatic
perihilar
extrahepatic

56
Q

What other name is sometimes used for perihilar cholangiocarcinoma?

A

1

57
Q

Define periampullary carcinoma?

A

tumors that surround the ampulla of vater

58
Q

Wha tare some risk factors for extrahepatic cholangiocarcinoma?

A

THey’re relatively uncommon tumors (Klatskin tumor)

typically in those over 50, more in males

anything causeing chronic cholangitis, infections with flukes, PSC, choledochal cysts (most have no diasease associations)

59
Q

Describe the clinical rpesentation in extraheptic cholangiocarcinoma.

A

usually painless jaundice secondary to obstruction

60
Q

What would the lab findings be for extrahepatic cholangiocarcioima?

A

typicall see a cholestatic injury pattern wiht increase in alkaline phosphatase and GGT

61
Q

Is the clinical presentation of extraheptic cholangiocarcinoma similar to the carcinoma of the head of the pancreas?

A

yes

62
Q

dEscribe how you would proceed ine valuating a patient (imaging and tissue diagnosis) for this.

A

Diagnosis requires demosntration of obstructin lesion and tissue biopsy or cytology indicating malignancy

can use DT, endoscopy with EUS or ERCP with cytology brushings and biopsy)

63
Q

Explain why the prognosis is so poor for thie type of tumor.

A

1

64
Q

Why is the prognosis for extrahepatic cholangiocarcinoma worse than for periampullary carcinoma?

A

1