Liver part three...cancer Flashcards by Tricia MD

What is HELLP syndrome?

Something to do with preeclampsia/eclampsia

Hemolysis
Elevated Liver enzymes
Low Platelets

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What is acute fatty liver of pregnancy?

Something to do with preeclampsia/eclampsia

Acute onset of liver dysfunction during pregnancy… can lead to death

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What is intrahepatic cholestasis of pregnancy?

Something to do with preeclampsia/eclampsia

Estrogenic hormones with biliary secretory defects –> mild increase conjugated bilirubin

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What liver abnormalities cay occur int bone marrow transplant patients?

Drug toxicity
Sinusoidal obstruction syndrome
Acute/chronic graft-vs-host disease

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What liver abnormalities can occur post liver transplant?

Preservation injury (oxygen radicals)
Anastomotic problems
Acute/chronic rejection

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What is hemangioma?

MOST COMMON benign neoplasm of the liver

Discrete red-blue hemorrhagic nodules

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What are simple cysts?

Single/small cluster

Rarely associated with polycystic kidney disease

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Polycystic liver disease?

Multiple cysts
Often associated with autosomal dominant polycystic kidney disease

Do NOT communicate with biliary system

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What is a choledochal cyst? What are some possible complications?

Congenital dilatation of the common bile duct

Biliary obstruction, stones, bile duct carcinoma

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What is Caroli’s disease?

Autosomal recessive cause of intrahepatic biliary dilatations that communicate with biliary tree

Associated with congenital hepatic fibrosis
Risk of cholangiocarcinoma

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What is Alagille syndrome?

Autosomal dominant cause of absence of bile ducts in portal tracts

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What is congenital hepatic fibrosis?

Autosomal recessive non-cirrhotic fibrotic liver disease of kids

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What is focal nodular hyperplasia?

Well-demarcated lesion composed of a proliferation of all liver parenchymal elements (central veins, hepatocytes, portal triads)

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What is seen with imaging of focal nodular hyperplasia?

Mass with a central fibrous scar of stellate configuration

Angiography shows a distinctive peripheral filling pattern

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What is nodular regenerative hyperplasia?

Diffuse non-fibrosing nodular hyperplasia of the liver

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What is a complication of nodular regenerative hyperplasia?

May develop portal hypertension

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What is hepatic adenoma?

Well-circumscribed benign neoplasm composed of well differentiated hepatocytes

NO portal triads or central veins

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Who is most likely to get hepatic adenoma?

Women of reproductive age on oral contraceptives

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What is the gross appearance of bile duct hamartoma?

Single…more commonly multiple…small white nodules

Mimics metastatic carcinoma

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What is the gross appearance of bile duct adenoma?

Usually solitary proliferation of bile ducts

Mimics metastatic carcinoma

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How are metastatic tumors diagnosed?

Obtain microscopic confirmation of lesions felt grossly to be liver metastases

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What are the risk factors for Hepatocellular Carcinoma (HCC)?

Hepatotrophic viruses
Cirrhosis
Hepatocarcinogens (toxin of aspergillus flavus)
Metabolic conditions (hereditary tyrosinemia; glycogen storage disease)

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What is the gross appearance of HCC?

Solitary mass…or with multiple nodules
Soft and hemorrhagic
Can metastasize to regional lymph nodes, diaphragm, lung, and/or bone

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Who gets HCC in the US? China?

US: men over 60yo
China: men at 40yo (high endemic HBV –> malignancy)

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How is HCC screened for?

Alpha-fetoprotein

What form of HCC has polygonal oncocytic tumor cells in nests and cords separated by lamellar fibrous stroma?

Fibrolamellar variant of HCC

What age group typically develops Fibrolamellar variant HCC? What is the 5 year survival?

Fibrolamellar affects people

What is cholangiocarcinoma?

Bile duct carcinoma | Virtually always adenocarcinomas

What are 5 risk factors for intrahepatic cholangiocarcinoma?

``` Thorotrast and liver flukes PSC Caroli's disease Congenital hepatic fibrosis Choledochal cysts ```

What is NOT associated with intrahepatic cholangiocarcinoma?

Cirrhosis or HBV

What is important when diagnosing intrahepatic cholangiocarcinoma

Adenocarcinoma metastatic to the liver HAS to be excluded

What is hepatoblastoma of the liver?

Malignancy composed immature hepatic elements (epithelial or mixed epithelial-immature stroma) Almost always occurs in infants Blastoma...like young/immature

What is angiosarcoma of the liver?

Malignancy of endothelial cells --> tumor composed of anastomosing vascular channels lined by atypical (malignant) endothelial cells

What is phyrgrian cap?

Folded fundus (phyrgrian cap. holy alliteration!)

What are the risk factors for developing cholesterol stones?

``` Four F's: Fat Female (from northern Europe, North/South America) Fertile (oral contraceptives) Forty ```

What are the risk factors for developing pigment stones?

From eastern Asia (bacterial and parasitic biliary infections) Hemolysis (increased unconjugated bilirubin)

What is used to look for gallstones?

Ultrasound

What percentage of people with gallstones have complications?

20-30%

What are 5 potential complications of gallstones?

``` Biliary "colic" Acute cholecystitis Chronic cholecystitis Choledocholithiasis (stones in common bile duct) Ascending cholangitis ```

What are 6 potential complications of acute cholecystitis?

``` Gallbladder perforation Bile peritonitis Acute cholangitis Sepsis Biliary-enteric fistula Gallstone ileus (stone gets trapped at ileocecal valve) ```

What is the most common cause of extra hepatic biliary obstruction?

Choledocholthiasis

What is chronic cholecystitis?

Gallbladder exhibits varying degrees of chronic lymphocytic inflammation and fibrosis

Porcelain gallbladder is associated with chronic cholecystitis, what is it?

Dystrophic calcification of the gallbladder wall

What is the gross appearance of cholesterolosis?

Yellow mucosal flecks

What is the gross appearance of a cholesterol polyp?

Yellow small polyps

What is the gross appearance of mucocele of the bladder?

Distended gallbladder with atrophic wall | Mucosa secretes a mucinous water fluid

What is adenoma of the gallbladder?

May have papillary appearance | BENIGN NEOPLASM

What is adenomyoma of the gallbladder?

Diverticulae with focal muscular hypertrophy of the gallbladder muscle wall at the fundus NOT a true neoplasm

What is a risk factor for developing gallbladder carcinoma?

Cholelithiasis

Why is the 5 year survival rate of gallbladder carcinoma so low?

Usually metastasizes to regional lymph nodes by onset of symptoms

What is the most common gallbladder carcinoma?

Adenocarcinoma

What are the three types (regions) of cholangiocarcinomas?

Intrahepatic Perihilar Distal extrahepatic

What is another name given to perihilar cholangiocarcinoma?

Klatskin tumor

What are periampullary carcinomas?

Tumors of the ampulla of Vater...include tumors arising from duodenal mucosa, pancreatic duct, and bile duct

What are 5 potential risk factors for extrahepatic cholangiocarcinomas?

``` Chronic ductal inflammation Bile stasis Parasitic infections PSC UC (often associated with PSC...pANCA...just another reminder) ```

What is the clinical presentation of extrahepatic cholangiocarcinomas?

Painless jaundice...secondary to obstruction

What previously talked about carcinoma also caused obstructive jaundice?

Pancreatic head carcinomas

What lab findings are seen with extrahepatic cholangiocarcinoma?

Cholestatic injury pattern with increase in ALP

What imaging studies can be done to see extrahepatic cholangiocarcinoma?

CT scan Endoscopy with EUS and FNA ERCP with cytology brushing

What is needed to diagnose extrahepatic cholangiocarcinoma?

Liver biopsy

Why does extrahepatic cholangiocarcinoma have such a poor prognosis?

Diffuse infiltrative tumor | Presents late??

Why is prognosis of periampullary carcinoma better than the prognosis for extrahepatic cholangiocarcinoma?

Periampullary presents sooner (per Kasey)