Theme 11 L3,4: Liver tumours, biliary and pancreatic pathology Flashcards

1
Q

What is an adenoma of the liver?

A

-a benign proliferation of epithelial cells (hepatocytes)

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2
Q

What are adenomas of liver cells often driven by?

A

exogenous steroids e.g OCP, anabolic steroids

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3
Q

If an adenoma ruptures, what complication could arise?

A

Haemoperitoneum

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4
Q

What condition can cause tiny white nodules on the liver?

A

adenoma of bile ducts

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5
Q

What is a von meyengerg complex?

A

multiple bile duct hamartomas or biliary microhamartomas

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6
Q

What are the two benign tumours of blood vessels of the liver?

A
  1. Hepatic haemangioma
  2. Focal nodular hyperplasia
    - young females
    - a regenerative, arterialised nodule
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7
Q

What is the most common malignant liver tumour?

A

hepatocellular carcinoma

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8
Q

What is the aetiology behind hepatocellular carcinoma?

A
  • cirrhosis
  • hepB/C
  • fatty liver disease or alcohol
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9
Q

What is secreted by malignant liver cells which can be detected by the blood and measured?

A

AFP (alpha feroprotein)

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10
Q

What is a cholangiocarcinoma?

A

malignant tumour of the bile duct cells

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11
Q

What are some causes of cholangiocarcinoma?

A

chronic inflammation - P.S.C, liver fluke

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12
Q

What is the main clinical feature of cholangiocarcinoma?

A

jaundice

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13
Q

What is an angiosarcoma?

A
  • aggressive malignant tumour of blood vessel

- strongly associated with toxins e.g vinyl chloride, thorotrast

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14
Q

Majority of liver tumours are a result of metastasis. What are the common sites of origin?

A

lung, breast, colon, pancreas

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15
Q

What is the aetiology of gall stones?

A
  • cholesterol, bile salts, bacterial growth + calcification

- slowly form a stone (calculus)

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16
Q

What are the risk factors for gallstones?

A

female
middle aged
over weight

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17
Q

What are the clinical features of gall stones?

A

crampy pain

- 80% asymptomatic

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18
Q

What are the complications of gallstones?

A
  • obstruction at neck - pain; obstruction
  • chronic cholecysitis
  • perforation
  • obstruction at pancreatic level - pancreatitis
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19
Q

What is chronic cholecystitis?

A
  • inflammation of the gall bladder
  • due to chemical or bacterial causes
  • over time causes fibrosis, ulceration of the gall bladder
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20
Q

For chronic cholecystitis:

  1. Clinical features?
  2. Diagnosis?
  3. Treatment?
A
  1. Pain (RUQ), fever, jaundice
  2. USS
  3. Cholecystectomy
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21
Q

How much of the pancreas is exocrine?

22
Q

What do exocrine glands of the pancreas secrete?

A

digestive enzymes - trypsin, lipase, phospholipase, elastase, amylase which require activation within the duodenum

23
Q

Which digestive enzyme of the pancreas is a marker of pancreatic inflammation?

24
Q

What is the endocrine function of the pancreas?

A

islets of langerhans secrete peptide hormones into the blood e.g insulin and glucagon

25
What is pancreatitis associated with?
injury to the exocrine parenchyma
26
What are the causes of acute pancreatitis?
-gallstones (50%) -alcohol (25%) rarer causes - vascular insufficiency, viral infections, hypercalcaemia -idiopathic
27
What is hereditary pancreatitis defined by?
- recurrent attacks of severe pancreatitis | - usually begins in childhood
28
Which two mutations are implicated in hereditary pancreatitis?
1. PRSS1 inherited mutations - autosomal dominant (inability to activate trypsin) 2. SPINK 1 Gene - autosomal recessive (autodigestion of pancreas)
29
What are the clinical features of acute pancreatitis?
- emergency - sudden onset of severe abdo pain radiating to the back - N&V - may be mild - raised serum amylase/lipase - persistent hypocalcaemia is a poor prognostic sign
30
What is the pathogenesis of acute pancreatitis?
-leakage and activation of enzymes -amylase released into blood mild pancreatitis: swollen gland with fat necrosis severe: swollen, necrotic gland with fat necrosis and haemorrhage -hypocalcaemia, hyperglycaemia, abscess formation and pseudocysts
31
What is: 1. Grey turner's sign 2. Cullen's sign
1. Haemorrhage into the sub cutaenous tissues of flank | 2. Peri umbilical haemorrhage
32
What are the complications of acute pancreatitis?
- shock - intravascular coagulopathy - haemorrhage - pseudocysts (collections of pancreatic juice secondary to duct rupture)
33
What is chronic pancreatitis?
- progressive inflammatory disorder in which parenchyma of pancreas is destroyed and replaced by fibrous tissue - irreversible destruction of the exocrine tissue, followed by destruction of the endocrine tissue
34
What are the clinical features of chronic pancreatitis?
- intermittent abdo pain, back pain and weight loss - malnutrition - diabetes
35
What are the causes of chronic pancreatitis? (TIGARO)
Toxic - alcohol, cigarettes, drugs, hypercalcaemia, hyperparathyroidism, infections Idiopathic Genetic - CFTR, PRSS1, SPINK-1 mutations Autoimmune Recurrent acute pancreatitis Obstruction of main duct - cancer, scarring
36
What are the complications of chronic pancreatitis?
- malabsorption of fat (lack of lipases) - steatorrhoea - impairment of fat soluble vit absorption - A, D, E and K - diarrhoea, weight loss, cachexia - diabetes - pseudocysts - stenosis of common bile duct/ duodenum - severe chronic pain
37
What is seen in patients with chronic pancreatitis associated with excess alcohol?
calculi
38
What is the most common type of pancreatic cancer?
pancreatic ductal adenocarcinoma
39
What are some causes of pancreatic ductal adenocarcinoma?
1. cigarette smoking 2. family history 3. nutritional and dietary factors rich in red meats, obesity, low physical activity, low intake of fruit and veg 4. other conditions - chronic pancreatitis, DM 5. alcohol
40
What is the difference between familial and hereditary pancreatic cancer?
Familial - pancreatic cancer in 2 or more first degree relatives Hereditary - due to an identified causative underlying germline mutation
41
Which hereditary cancer syndromes can cause pancreatic cancer?
``` KRAS mutations first then FAMMM (p16) then p53, DPC4, BRCA2 -TP53 mutations in Li-Fraumeni syndrome and STK11 mutations for Peutz-Jeghers syndrome then PanIN ```
42
What is PanIN?
Pancreatic intra epithelial neoplasia | PanIN (1,2,3)
43
Where are 70% of pancreatic cancers located?
head of pancreas
44
What is a desmoplastic response?
intense non-neoplastic reaction by host to presence of tumour forming firm fibrous tissue surrounding the tumour
45
why does pancreatic cancer have such a high mortality?
- early cancer is silent - present for at least a decade before detected - non specific symptoms - epigastric pain, radiating to back
46
What is Trousseau's syndrome?
migratory thrombophlebitis - poor prognostic factor of pancreatic carcinoma
47
What is Courvoisier's sign?
palpable gall bladder without pain - poor prognostic factor of pancreatic carcinoma
48
What is a pancreatic neuroendocrine tumour?
- uncommon pancreatic neoplasm derived from islet cells - benign or malignant - often occult (not associated with concerning signs and symptoms) as higher incidence in autopsy
49
What are the risk factors for pancreatic neuroendocrine tumours?
M=F Risk factors - smoking, family history of cancer, alcohol, obesity, diabetes -increased risk in MEN-1, NF-1, VHL
50
What are some examples of neuroendocrine neoplasms and what cell type and clinical findings are associated to each?
1. Insulinoma B cell Clinical finding - hypoglycaemia 2. Glucagonoma A cell 3. Somatostatinoma D cell clinical finding: hypochlorhydria some can be non-functioning