Liver Path: Cholestatic/Neoplastic Disease Flashcards
What are causes of steatosis? (4)
Alcoholic liver disease
NAFLD
Ischemia
Inherited disorders: FAO defects, mitochondriopathies, galctosemia, fructose intolerance, CF, cholesterol storage disorder
Describe the histology of alcoholic liver disease (2)
Steatosis with pericentral sinusoidal (arachnoidal/chicken wire) fibrosis
Mallory denk bodies (balloon appearance with eosinophilic hyaline bodies)
NAFLD: describe the prevalence and histology
Most common cause of chronic liver disease in US (3-5% population)
Histological features in adults overlap with alcoholic liver disease
Describe the difference between hemochromatosis and hemosiderosis
Hemochromatosis: due to gene mutation in HFE, transferrin receptor, hepcidin or HJV– it is hereditary
Hemosiderosis is secondary hemochromatosis due to other causes
Describe the causes of hemosiderosis (5)
Parenteral iron overload, Thalassemia
Increased oral intake
Chronic liver disease
Neonatal hemochromatosis
Describe histology for hereditary hemochromatosis
Using prussian blue stain, can see iron deposition in hepatocytes (it is golden-brown)
Also observe iron deposition in pancreas, heart, joints, endocrine organs
Describe histology of hemosiderosis
Iron accumulation mainly occurs in kuppfer cells
What is cause of neonatal hemochromatosis?
Prenatal onset: in-utero allimmune reaction of mother with formation of anti-liver antibodies resulting in liver/renal failure
Describe the intrahepatic causes of cholestasis (5)
Diffuse hepatocellular disease (sepsis, hepatitis, cirrhosis)
Canalicular membrane changes (drugs/pregnancy)
Genetic defects in cholestatic transporters (Dubin-Johnson, Rotor)
Primary biliary cirrhosis
Ductopenia
Describe extra hepatic causes of cholestasis (4)
Bile duct stones
Intra/extra biliary tumors
Sclerosing cholangitis
Biliary atresia
Compare and contrast primary biliary cirrhosis vs primary sclerosing cholangitis:
Age, gender, associated conditions, serology, radiology, duct lesion
Do it do it
Describe PBC: Age, gender, associated conditions, serology, radiology, duct lesion
Age: median=50yo Gender: 90% female Associated conditions: Sjogren, thyroid disease Serology: AMA, ANA, ANCA Radiology: normal Duct lesion: Inflammatory destruction
Describe PSC: Age, gender, associated conditions, serology, radiology, duct lesion
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
Describe pathophysiology of primary biliary cirrhosis
Inflammatory destruction of interlobular bile ducts with granulomas leading to cirrhosis with ductopenia
Describe histology of PSC
Fibrous obliterative ductal lesions
Onion skinning fibrosis around destroyed bile duct
What are causes of ductopenia in adults? (3)
Immune: PBC, PSC, sarcoidosis, rejection, GVHD
Secondary: mechanical, drugs
Idiopathic
What are causes of ductopenia in pediatric populations? (4)
Syndromatic: Alagille syndrome
Idiopathic
Metabolic: A1AT deficiency
Secondary: biliary atresia
What is alagille syndrome?
Autosomal dominant disorder leading to pathology in liver, heart, skeleton, eye, face, kidney, vasculature
What are causes of obstructive cholestasis? (4)
Cholelithiasis (gallstones)
Malignancy of biliary tree of pancreas head
Strictures from surgery
Biliary atresia (children)
What are histological findings of extra hepatic bile duct obstruction? (4)
Portal fibrous expansion
Bile duct proliferation
Bile plugs
Feathery degeneration of hepatocytes
What histological finding occurs in ascending cholangitis due to gallstones?
Increased neutrophils
What are pathology findings in late duct obstruction? Histological and gross
Biloma: a bile filled cyst in liver
Biliary cirrhosis: due to extravasation of bile within liver
Hepatic Infarction: histology
Coagulative necrosis of hepatocytes with hyperemic rim on edge of necrosis
Can also observe infarct of major ducts of biliary tree
Describe causes of portal venous obstruction: both extrahepatic (4) and intrahepatic (2)
Extrahepatic: neonatal umbilical vein catheterization, intraabdominal sepsis, hypercoagulable disorders, pancreatitis
Intrahepatic: schistosomiasis, obliterative portal venopathy
What is the cause of Budd Chiari Syndrome?
What are the symptoms? (3)
Outflow obstruction due to hepatic vein thrombosis
Symptoms of Budd Chiari include hepatomegaly, ascites and liver dysfunction
What is sinusoidal obstruction syndrome (aka veno-occlusive disease)?
Endothelial injury to sinusoids and terminal hepatic venules
Observed in patients post stem cell tx and receiving chemo
What are histological findings in sinusoidal obstruction syndrome? (3)
Centrilobular congestion
Hepatocellular necrosis
Obliteration of small hepatic veins
How does liver appear in CHF? (3)
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
What is the cause of echninococcal/hyatid cysts?
What is concern during removal?
Larval stages of tapeworm
Surgical removal risks rupture which can result in fever, anaphylaxis
What are other types of cysts observed in liver? (2)
Simple cysts– bile duct cyst
Polycystic liver disease
What are the two types of vascular tumors in liver?
How do they present?
Carvernous hemangioma==>most common liver tumor in adults; often asymptomatic or produces abd pain
Infantile hemangioendothelioma==>most common liver tumor in children
What is hepatocellular adenoma associated with? (2)
OCP Metabolic diseases (i.e glycogen storage disease)
Describe focal nodular hyperplasia–
Epidemiology (prevalence, demographic)
Gross appearance
Most frequent solid lesion
3% of population, young females
Surrounding liver is normal; there is a central stellate scar
What are the types of malignant epithelial liver tumors? (3)
HCC
Cholagniocarcinoma
Hepatoblastoma
Describe the epidemiology of HCC: prevalence, geography, RFs
HCC=5% all cancers
Highest incidence in asian countries with high HBV prevalence
RFs: HBV, HCV, Aflatoxin, alcohol, metabolic diseases…anything that causes cirrhosis
What are the prognostic factors for HCC? (4)
What is the prognosis for HCC?
Vascular invasion
Differentiation
Number/size of nodules
Extra hepatic spread (IVC, lungs, regional lymph nodes)
Prognosis is terrible (
What is precursor for HCC?
Dysplastic lesions
What is fibrolamellar carcinoma?
A variant of HCC (makes up 5%)
Typically appears
Hepatoblastoma: how does it present? what is prognosis?
Presents with asymptomatic abdominal mass in children
Better prognosis than HCC
