Oncology 6 - Liver And Pancreas

Front 1

What are the most common benign epithelial tumors of liver?

Back 1

1. Focal nodular hyperplasia

Front 2

What is focal nodular hyperplasia?
How common is it - and in who?
And what is it difficult to distinguish it from?

Back 2

A benign epithelial pseudotumor (hyperplasia) of hepatocytes - occuring in young women - typically taking oral contraceptives.

Difficult to distinguish from:
- Liver cell adenocarcinoma
- Hepatocellular carcinoma

Therefore it is commonly resected.

Front 3

What is the most common benign tumor of liver?

What are their origin?

Back 3

1. Cavernous hemangioma (mesenchymal)
2. Focal nodular hyperplasia (epithelial)

Front 4

What is the second most common benign epithelial tumor of liver?

Back 4

Hepatocellular adenoma (think it's called liver cell adenoma…)

Front 5

What is a cavernous hemangioma?

Back 5

A benign tumor of dilated blood vessels - form a red nodule on skin - or anywhere else in the body.

Front 6

Cavernous hemangioma of liver is associated with which syndrome?

What is this syndrome?

Back 6

Kasabach-Merritt syndrome

A consumptional thrombopathy, with thrombocyte consumption in caverns - leading to consumtional thrombocytopenia and spontaneous bleedings.

Front 7

What is the most common tumor of liver?

Back 7

Hematogenous metastasis from somewhere else - especially when there is no liver cirrhosis

Front 8

Where do the secondary cancers of liver usually spread from?

Back 8

1. Colon
2. Lung
3. Breast
4. Leukemic infiltration (hepatomegaly)

Front 9

What is the most common primary epithelial carcinomas of liver?

Back 9

1. Hepatocellular carcinoma
2. Cholangiogenic carcinoma
3. Hepatoblastoma

Front 10

What are the most common primary non-epithelial carcinomas of liver? (quite rare)

Back 10

1. Epithelioid hemangioendothelioma
2. Hemangiosarcoma
3. Primary hepatic lymphoma

Front 11

What is hepatocellular carcinoma?

Back 11

A malignant epithelial tumor of hepatocytes

Front 12

Etiological factors of heptocellular carcinoma?

Back 12

1. Chronic hepatitis
2. Viral hepatitis (B/C)
3. Chronic alcoholism --> Cirrhosis
4. Drugs
5. Diet

Front 13

What usually always precedes a hepatocellular carcinoma?

Back 13

Cirrhosis of the liver - develops from high grade dysplastic nodules in cirrhotic liver, usually.

Since there is high genomic instability.

Front 14

What markers are elevated in hepatocellular carcinoma?

Back 14

Alpha-fetoprotein

Front 15

Common metastasis locations of hepatocellular carcinoma?

Back 15

- Lungs
- Bones
- Lymph nodes

=> Very easy vascular invasion

Front 16

How does hepatocellular carcinoma look in LM?

Back 16

- Distorsions of normal liver architecture.
- Liver plates are widened and pseudoacinar structres found

1. Well differentiated with hepatic cords growing in trabecules or glands
2. Poorly differentiated with mostly multinuclear anaplastic giant cells

Front 17

What is a typical carcinogen for hepatocellular carcinoma of liver?

Back 17

Aflatoxin from aspergillus flavus

Front 18

What is cholangiogenic carcinoma of liver?

What is it's characteristics?

Back 18

Carcinoma of intraepithelial bile ducts of liver

Grow in multiple nodules, with highly infiltrative growth.

Front 19

What is famous etiological agent of cholangiogenic carcinoma of liver?

Back 19

Thorotrast - contrast medium used earlier in X-rays - common in CZ

Front 20

What is a hepatoblastoma?

Back 20

A rare, pediatric malignant tumor of immature liver cells - grow to become fetal or adult liver cells or lymph cells

Front 21

What are the most common mesenchymal liver malignancies again?

Back 21

1. Hemangiosarcoma of liver
2. Epithelioid hemangioendothelioma
3. Primary hepatic lymphoma

Front 22

What is the famous etiological carcinogen causing hemangiosarcoma of liver?

Back 22

PVC (poly vinyl chloride) from the tubing industry

Front 23

What is a epithelioid hemangioendothelioma?

Back 23

A intermediate between hemangioma and angiosarcoma

May also be called low grade hemangiosarcoma

Front 24

What are 2 typical benign tumors of biliary system?

Back 24

1. Cholesterol polyps / strawberry gallbladder
2. Adenomyoma & adenomyomatosis

Front 25

What are the cholesterol polyps?

Back 25

High lipid content of bile leads to accumulation of lipids in mucosa of gall-bladder macrophages : Becomes 'lipophages'.

And this looks like a strawberry actually!

Front 26

What are adenomyoma and adenomyomatosis?

Back 26

Hyperplasia of gall bladder mucosa and muscular layer (it is a muscular sac, storing up to 100ml bile)

Front 27

What is the most common pure gall bladder cancer?

Back 27

Adenocarcinoma of gall bladder

Front 28

Typical patient who gets gall bladder carcinoma?

What are the risk factors?

Back 28

Female, fat and fertile - the same risk factors as gall stone formations.

Contributing also is gallstones - since they chronically irritate the wall and cause pressure atrophy, higher genomic instability and regeneration attempts.

Front 29

What is typical tumor of intrahepatic bile ducts?
How does it look?

Back 29

Cholangiogenic carcinoma - of PVC remember!

Tumor cells are forming glandular structures, in a dense sclerotic stroma due to desmophilic reaction.

Front 30

What is typical tumor of extrahepatic bile ducts?

Leading to what symptoms and what serological markers?

Are they malignant?

Back 30

1. Carcinoma of Vater's papilla
2. Confluence of cystic and hepatic duct

Lead to early jaundice
Elevated alkaline phosphatase and aminotransferases

Not malignant - only obstruct the bile ducts

Front 31

Most common benign tumors of exocrine pancreas?

Back 31

1. Adenomas (IPMNs)
2. Cystadenomas

Front 32

2 types of cystadenomas of pancreas?

Back 32

1. Sereous - not dangerous
2. Mucinous - premalignant!

Front 33

What are the 2 premalignant tumors of exocrine pancreas?

Back 33

1. Intraductal papillary mucinous neoplasm (IPMNs)
2. Mucinous cystadenoma / cyst neoplasia

Front 34

How do we measure degree of atypia and potential cancer in pancreas?

Back 34

Use PanIN: Pancreatic intraepithelial neoplasia

Same as anywhere else...

Front 35

IPMNs - what are they, where do they arise and in who?

Back 35

They are intraductal papillary mucinous neoplasias - a mucin producing intraductal neoplasms…

Occur in women most often and arise in the main pancreatic ducts in head of pancreas - thus also obstructing the bile duct causing jaundice.

Show different degrees of dysplasia - and may develop into invasive carcinoma.

Front 36

Majority of malignant tumors of exocrine pancreas are?

What do they arise from?

Back 36

Ductal adenocarcinomas

Arise either from:
1. Primary of pancreatic duct
2. IPMN

Front 37

What is difference in pancreatic carcinoma of head and body/tail?

Back 37

Head:
- Discovered earlier due to compression of bile duct and jaundice

Body/tail:
- Discovered late, since no symptoms, so it has grown much larger.

Front 38

What are the other clinical symptoms of a pancreatic carcinoma?

Back 38

Weight loss
Abdominal pain (perineural spread)
Thrombophlebitis

Front 39

How is thrombophlebitis migrans connected to pancreatic carcinoma?

What is it also called?

Back 39

A paraneoplastic syndrome leads to thrombosis at distant sites - for example in femoral vein

Called 'Trousseau sign'

Front 40

What is thrombophlebitis migrans?

Back 40

Vein inflammation associated with tumor - occuring repeatedly at different locations.

Often associated with pancreatic carcinoma

Front 41

What are the risk factors of pancreatic carcinoma?

Back 41

- Male 5-7th decade
- Alcohol, smoking, high fat diet
- Chronic pancreatitis

Front 42

How is pancreatic carcinoma…
1. Macroscopically
2. Microscopically

Back 42

Macro
- Mimic pancreatitis

Micro
- Small gland adenocarcinoma with atypical shapes
- Spread perineurally (pain)
- Invade vessels easy

Front 43

Pancreatic adenocarcinoma spreads usually to?

Back 43

Regional lymph nodes and hematogenous

Front 44

What is the name of the sign of pancreatic adenocarcinoma - where we have a non-neoplastic enlargement of the gallbladder?

Back 44

Courvoisier's sign

Front 45

A 15 y old boy from America came up with a new test for pancreatic adenocarcinoma. Which tumor marker did he find that correlated to 90% of the cancers?

What other cancer also gives overexpression of this?

Back 45

Mesothelin

Overexpressed in:
- Mesothelioma
- Ovarian adenocarcinoma
- Pancreatic adenocarcinoma

Front 46

What other types of malignant neoplasias do we have in pancreas?

Back 46

1. Ductal adenocarcinoma (95%)
2. Acinar adenocarcinoma (5%)
3. Solid pseudopapillary neoplasia
4. Neuroendocrine tumors

Front 47

What is solid pseudopapilary neoplasia?

Back 47

A solid, pseudopapillary lesion of intermediate malignancy - occuring in young women around 20

Front 48

What is the prevalence of benign and malignant tumors of ENDOCRINE pancreas?

Back 48

Benign
- Most common - asymptomatic unless they secrete excess hormone

Malignant
- 10% only - clinical effects also vary with hormone produced

Front 49

Under what classification does endocrine tumors of pancreas occur?

Back 49

Under neuroendocrine tumors !!

Front 50

If Steiner asks - what is the name of benign tumors of endocrine pancreas?

Back 50

Mesidioma ???

Front 51

If tumor comes from B-cell of pancreas - it is called what and leads to?

Back 51

Insulinoma - leads to hypoglycemia

Front 52

If tumor comes from A-cells of pancreas - it is called what and leads to?

Back 52

Glucagonomas - leads to hyperglycemia and diabetes!

Front 53

If tumor comes from d-cells of pancreas - it is called what and leads to?

Back 53

Somatostatinoma - also leads to diabetes

Since if both glucagon & insulin are stopped - there are other hormones that counter insulin and favours hyperglycemia! (next q)

Front 54

Which hormones counteract insulin?

Back 54

1. Glucagon
2. Growth hormone
3. Glucocorticoids

Front 55

If there is some ectopic production of gastrin from pancreas - it is called what? (2 names)

What does it lead to?

Back 55

Gastrinoma or more famously Zollinger-Ellison syndrome - found in the head of pancreas usually

Leads to multiple peptic ulcers

Front 56

Other hormones that may be secreted by pancreatic tumors and what do they lead to?

Back 56

1. Serotonin --> Carcinoid syndorme

2. ACTH --> Cushing's syndrome

Front 57

What is carcinoid syndrome?

Back 57

Syndrome occuring in carcinoid tumors (serotonin-producing) with symptoms including:

- Flushing
- Diarrhea
- Bronchoconstriction
- Heart failure

(latter 2 is less frequent)

Front 58

Pancreatic endocrine neoplasms is a part of the 'Multiple Endocrine Neoplasia syndromes' (MENs)

Which MEN is pancreatic endocrine neoplasms part of?

Back 58

MEN1

Front 59

What does MEN1 consist of?

Back 59

1. Parathyroid adenoma
2. Pancreatic endocrine neoplasms
3. Pituitary adenomas - especially prolactinomas

Front 60

What is the cause of MEN1?

Back 60

A mutation of MEN1 gene - which encodec 'menin' - a nuclear protein

Not more is known?

Front 61

What does MEN2 consist of?

Back 61

1. Medullary carcinoma of thyroid
2. Pheochromocytoma

Front 62

MEN1 is also called?

Back 62

Wermer syndrome

Front 63

MEN2 is also called?

Back 63

Sipple syndrome

Front 64

What is the cause of MEN2?

Back 64

Mutatino in activating ret oncogene on chromosome 10 - which encodes a cell surface receptor with tyrosine kinase activity

Front 65

What is BTW the 4th cell of pancreatic islets?

Back 65

Pancreatic polypeptide cell (PP) - found in highest concentration at head of pancreas