Week 4: Liver Neoplasms and Autoimmune Hepatitis/Cholangiopathies Flashcards

1
Q

benign hepatocellular neoplasms which may be detected from abdominal pain but are often detected incidentally on imaging

A

Hepatocellular Adenomas (HCA)

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2
Q

What molecular activation is most commonly associated with Hepatocellular Adenomas (HCA)

A

Beta-catenin

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3
Q

ID these features for b-catenin activated Hepatocellular Adenomas (HCA)

A
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4
Q

ID

A

b-catenin activated Hepatocellular Adenomas (HCA)

hepatocytes look relatively normal except that they are forming abnormal tube-like structures called pseudoacini (red circles)

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5
Q

ID

A

b-catenin activated Hepatocellular Adenomas (HCA)

increased cell atypia characterized by nuclei that are slightly increased in size relative to their cytoplasm (yellow arrows)

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6
Q

ID

A

b-catenin activated Hepatocellular Adenomas (HCA)

Nuclear B-catenin staining on IHC (yellow arrows). Normal nuclei appear blue

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7
Q

ID

A

Nuclear B-catenin staining on IHC (yellow arrows). Normal nuclei appear blue

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8
Q

ID

A

Hepatocellular Carcinoma (HCC)

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9
Q

What are common mutations in Hepatocellular Carcinoma (HCC)

A
  • TERT (telomerase transcriptase) promoter
  • CTNNB1 gene
  • beta-catenin gene
  • TP53 inactivations
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10
Q

ID

A

Hepatocellular Carcinoma (HCC)

distorted versions of normal architecture, including large pseudoacinar spaces (yellow arrow)

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11
Q

ID

A

Hepatocellular Carcinoma (HCC)

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12
Q

ID and staining

A

Normal Liver and Reticulin stain

terminal hepatic venule / central vein (orange arrow)

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13
Q

ID and staining

A

Hepatocellular Carcinoma (HCC) and Reticulin stain

fragmented/lost fibers (red elipses) and terminal hepatic venule / central vein (orange arrow)

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14
Q

HCC accounts for approximately 5.4% of all cancers
worldwide and is one of the most common cancers in
geographic regions with high rates of _____

A

Hepatitis B

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15
Q

Risk factors for Cholangiocarcinoma

A
  • Primary sclerosing cholangitis
  • liver fluke infection
  • hepatolithiasis
  • HBV and HCV
  • MAFLD
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16
Q

ID

A

Cholangiocarcinoma (CAC)

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17
Q

What is a cholangiocarcinoma called when it occurs at the bifurcation of the left/right hepatic ducts

A

Klatskin tumor

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18
Q

This biopsy was taken from the bile ducts

ID

A

Cholangiocarcinoma (CAC)

impossible to distinguish cholangiocarcinoma from metastatic adenocarcinomas by histology alone

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19
Q

ID

A

Cholangiocarcinoma (CAC)

impossible to distinguish cholangiocarcinoma from metastatic adenocarcinomas by histology alone

20
Q

ID

A

Cholangiocarcinoma (CAC)

tumor (yellow arrow) in contact with and tracking along the nerve (orange arrow) forming a wreathlike pattern is termed perineural invasion

21
Q

What are the four most common sources of liver metastases

A
  • Colon
  • Breast
  • Lung
  • Pancreas
22
Q

ID

A

Liver Metastases

They are 18-40x more common than primary liver neoplasms

23
Q

ID

A

Autoimmune Hepatitis (AIH)

interface activity, where the inflammation is migrating away from the portal vein (red elipse)

24
Q

ID

A

Autoimmune Hepatitis (AIH)

A focus of lobular hepatitis with prominent plasma cells typical for this disease

25
Q

What 4 things do you use to classify Autoimmune Hepatitis (AIH)

A
  • Autoantibodies
  • IgG
  • Liver Histology
  • Absence of viral hepatitis
26
Q

Is a chronic cholestatic disease in which there is progressive autoimmune destruction of the intrahepatic bile ducts by T lymphocytes

A

Primary biliary cholangitis (PBC)

27
Q

What lab values are usually elevated in Primary biliary cholangitis (PBC)

A
  • ALK/P
  • GGT
  • Bilirubin
28
Q

What antibodies are present 90-95% of the time in Primary biliary cholangitis (PBC)

A

Antimitochondrial antibodies (AMA) +

29
Q

What is treatment for Primary biliary cholangitis (PBC)

A

Ursodeoxycholic acid (Ursodiol)

30
Q

ID

A

Primary biliary cholangitis (PBC)

greenish yellow discoloration of the cirrhotic nodules (cyan arrows)

31
Q

ID

A

Primary biliary cholangitis (PBC)

the bile duct (yellow circle) is being surrounded by dense portal inflammation, a florid duct lesion - often with granulomas (yellow arow)

32
Q

ID

A

Primary biliary cholangitis (PBC)

the bile duct (yellow circle) is being surrounded by dense portal inflammation, a florid duct lesion - often with granulomas (yellow arow)

33
Q

Progressive inflammation and scarring of biliary tree (both intrahepatic and extrahepatic)

A

Primary Sclerosing Cholangitis (PSC)

34
Q

What is associated with Primary sclerosing Cholangitis (PSC)

A

Ulcerative Colitis

Inflammatory Bowel Disease (IBD)

35
Q

What lab values are usually elevated in Primary Sclerosing cholangitis (PSC)

A
  • ALK/P
  • GGT
  • Bilirubin
36
Q

Having Primary Sclerosing Cholangitis (PSC) puts you at an increased risk for what cancer?

A

Cholangiocarcinoma (CAC)

37
Q

ID

A

Primary Sclerosing Cholangitis (PSC)

imaging evidence of biliary stricturing (closed white arrows) which occurs as a result of the progressive inflammation and scarring of the biliary tract along with dilation of the biliary traction (open white arrow) proximal to the stricturing

38
Q

ID

A

Primary Sclerosing Cholangitis (PSC)

the injured bile ducts, which become so distorted and overwhelmed by inflammation (red arrow) that they can be barely recognizable

39
Q

ID

A

Primary Sclerosing Cholangitis (PSC)

the injured bile ducts, which become so distorted and overwhelmed by inflammation (red arrow) that they can be barely recognizable

40
Q

ID

A

Primary Sclerosing Cholangitis (PSC)

periductal ‘onion-skin’ fibrosis (red arrow)

41
Q
A

Primary Sclerosing Cholangitis (PSC)

periductal ‘onion-skin’ fibrosis (red arrow) as well as eventually complete fibrous obliteration of bile ducts ‘tombstone scars’ (cyan arrow)

42
Q

What is associated with Primary Biliary Cirrhosis (PBC)

A

Sjögren syndrome

43
Q

What antibodies are present 65% of the time in Primary Sclerosing Cholangitis (PSC)

A

p-ANCA

MPO+ and also associated with microscopic polyangitis and Churg-Straus Syndrome

44
Q

What is the primary demographic of people who get Primary Biliary Cirrhosis (PBC)

A

90% female

45
Q

What is the primary demographic of people who get Primary Sclerosis Cholangitis (PSC)

A

70% male