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 the organ and staining

A

Liver; Silver stain

terminal hepatic venule (orange arrow) and A LOT of bile canaliculi in black (blue arrows pointing to 3 of many)

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13
Q

ID and staining

A

Normal Liver and Reticulin stain

terminal hepatic venule / central vein (orange arrow)

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14
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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15
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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16
Q

Risk factors for Cholangiocarcinoma (CAC)

A
  • Primary Sclerosing Cholangitis (PSC)
  • liver fluke infection
  • hepatolithiasis
  • HBV and HCV
  • MAFLD
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17
Q

ID

A

Cholangiocarcinoma (CAC)

Non-cirrhotic liver (yellow arrow)

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18
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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19
Q

This biopsy was taken from the bile ducts

ID

A

Cholangiocarcinoma (CAC)

impossible to distinguish cholangiocarcinoma from metastatic adenocarcinomas by histology alone

20
Q

ID

A

Cholangiocarcinoma (CAC)

impossible to distinguish cholangiocarcinoma from metastatic adenocarcinomas by histology alone

21
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

22
Q

What are the four most common sources of liver metastases

A
  • Colon
  • Breast
  • Lung
  • Pancreas
23
Q

ID

A

Liver Metastases

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

24
Q

ID

A

Autoimmune Hepatitis (AIH)

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

25
ID
Autoimmune Hepatitis (AIH) ## Footnote A focus of lobular hepatitis with prominent plasma cells typical for this disease
26
What 4 things do you use to classify Autoimmune Hepatitis (AIH)
* Autoantibodies * IgG * Liver Histology * Absence of viral hepatitis
27
Is a chronic cholestatic disease in which there is progressive autoimmune destruction of the intrahepatic bile ducts by T lymphocytes
Primary biliary cholangitis (PBC)
28
What lab values are usually elevated in Primary biliary cholangitis (PBC)
* ALK/P * GGT * Bilirubin
29
What antibodies are present 90-95% of the time in Primary biliary cholangitis (PBC)
Antimitochondrial antibodies (AMA) +
30
What is treatment for Primary biliary cholangitis (PBC)
Ursodeoxycholic acid (Ursodiol)
31
ID
Primary biliary cholangitis (PBC) ## Footnote greenish yellow discoloration of the cirrhotic nodules (cyan arrows)
32
ID
Primary biliary cholangitis (PBC) ## Footnote the bile duct (yellow circle) is being surrounded by dense portal inflammation, a florid duct lesion - often with granulomas (yellow arow)
33
ID
Primary biliary cholangitis (PBC) ## Footnote the bile duct (yellow circle) is being surrounded by dense portal inflammation, a florid duct lesion - often with granulomas (yellow arow)
34
Progressive inflammation and scarring of biliary tree (both intrahepatic and extrahepatic)
Primary Sclerosing Cholangitis (PSC)
35
What is associated with Primary sclerosing Cholangitis (PSC)
Ulcerative Colitis ## Footnote Inflammatory Bowel Disease (IBD)
36
What lab values are usually elevated in Primary Sclerosing cholangitis (PSC)
* ALK/P * GGT * Bilirubin
37
Having Primary Sclerosing Cholangitis (PSC) puts you at an increased risk for what cancer?
Cholangiocarcinoma (CAC)
38
ID
Primary Sclerosing Cholangitis (PSC) ## Footnote 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
39
ID
Primary Sclerosing Cholangitis (PSC) ## Footnote the injured bile ducts, which become so distorted and overwhelmed by inflammation (red arrow) that they can be barely recognizable
40
ID
Primary Sclerosing Cholangitis (PSC) ## Footnote the injured bile ducts, which become so distorted and overwhelmed by inflammation (red arrow) that they can be barely recognizable
41
ID
Primary Sclerosing Cholangitis (PSC) ## Footnote periductal 'onion-skin' fibrosis (red arrow)
42
ID
Primary Sclerosing Cholangitis (PSC) ## Footnote periductal 'onion-skin' fibrosis (red arrow) as well as eventually complete fibrous obliteration of bile ducts 'tombstone scars' (cyan arrow)
43
What is associated with Primary Biliary Cholangitis (PBC)
Sjögren syndrome
44
What antibodies are present 65% of the time in Primary Sclerosing Cholangitis (PSC)
p-ANCA ## Footnote MPO+ and also associated with microscopic polyangitis and Churg-Straus Syndrome
45
What is the primary demographic of people who get Primary Biliary Cirrhosis (PBC)
90% female
46
What is the primary demographic of people who get Primary Sclerosis Cholangitis (PSC)
70% male