Liver part three...cancer Flashcards
What is HELLP syndrome?
Something to do with preeclampsia/eclampsia
Hemolysis
Elevated Liver enzymes
Low Platelets
What is acute fatty liver of pregnancy?
Something to do with preeclampsia/eclampsia
Acute onset of liver dysfunction during pregnancy… can lead to death
What is intrahepatic cholestasis of pregnancy?
Something to do with preeclampsia/eclampsia
Estrogenic hormones with biliary secretory defects –> mild increase conjugated bilirubin
What liver abnormalities cay occur int bone marrow transplant patients?
Drug toxicity
Sinusoidal obstruction syndrome
Acute/chronic graft-vs-host disease
What liver abnormalities can occur post liver transplant?
Preservation injury (oxygen radicals)
Anastomotic problems
Acute/chronic rejection
What is hemangioma?
MOST COMMON benign neoplasm of the liver
Discrete red-blue hemorrhagic nodules
What are simple cysts?
Single/small cluster
Rarely associated with polycystic kidney disease
Polycystic liver disease?
Multiple cysts
Often associated with autosomal dominant polycystic kidney disease
Do NOT communicate with biliary system
What is a choledochal cyst? What are some possible complications?
Congenital dilatation of the common bile duct
Biliary obstruction, stones, bile duct carcinoma
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
What is Alagille syndrome?
Autosomal dominant cause of absence of bile ducts in portal tracts
What is congenital hepatic fibrosis?
Autosomal recessive non-cirrhotic fibrotic liver disease of kids
What is focal nodular hyperplasia?
Well-demarcated lesion composed of a proliferation of all liver parenchymal elements (central veins, hepatocytes, portal triads)
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
What is nodular regenerative hyperplasia?
Diffuse non-fibrosing nodular hyperplasia of the liver
What is a complication of nodular regenerative hyperplasia?
May develop portal hypertension
What is hepatic adenoma?
Well-circumscribed benign neoplasm composed of well differentiated hepatocytes
NO portal triads or central veins
Who is most likely to get hepatic adenoma?
Women of reproductive age on oral contraceptives
What is the gross appearance of bile duct hamartoma?
Single…more commonly multiple…small white nodules
Mimics metastatic carcinoma
What is the gross appearance of bile duct adenoma?
Usually solitary proliferation of bile ducts
Mimics metastatic carcinoma
How are metastatic tumors diagnosed?
Obtain microscopic confirmation of lesions felt grossly to be liver metastases
What are the risk factors for Hepatocellular Carcinoma (HCC)?
Hepatotrophic viruses
Cirrhosis
Hepatocarcinogens (toxin of aspergillus flavus)
Metabolic conditions (hereditary tyrosinemia; glycogen storage disease)
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
Who gets HCC in the US? China?
US: men over 60yo
China: men at 40yo (high endemic HBV –> malignancy)
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 < 35yo
Better 5 year survival than other HCC, but late recurrences are common
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)
