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?

A

85%

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?

A

amylase

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
Q

What is pancreatitis associated with?

A

injury to the exocrine parenchyma

26
Q

What are the causes of acute pancreatitis?

A

-gallstones (50%)
-alcohol (25%)
rarer causes - vascular insufficiency, viral infections, hypercalcaemia
-idiopathic

27
Q

What is hereditary pancreatitis defined by?

A
  • recurrent attacks of severe pancreatitis

- usually begins in childhood

28
Q

Which two mutations are implicated in hereditary pancreatitis?

A
  1. PRSS1 inherited mutations - autosomal dominant (inability to activate trypsin)
  2. SPINK 1 Gene - autosomal recessive (autodigestion of pancreas)
29
Q

What are the clinical features of acute pancreatitis?

A
  • 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
Q

What is the pathogenesis of acute pancreatitis?

A

-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
Q

What is:

  1. Grey turner’s sign
  2. Cullen’s sign
A
  1. Haemorrhage into the sub cutaenous tissues of flank

2. Peri umbilical haemorrhage

32
Q

What are the complications of acute pancreatitis?

A
  • shock
  • intravascular coagulopathy
  • haemorrhage
  • pseudocysts (collections of pancreatic juice secondary to duct rupture)
33
Q

What is chronic pancreatitis?

A
  • 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
Q

What are the clinical features of chronic pancreatitis?

A
  • intermittent abdo pain, back pain and weight loss
  • malnutrition
  • diabetes
35
Q

What are the causes of chronic pancreatitis? (TIGARO)

A

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
Q

What are the complications of chronic pancreatitis?

A
  • 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
Q

What is seen in patients with chronic pancreatitis associated with excess alcohol?

A

calculi

38
Q

What is the most common type of pancreatic cancer?

A

pancreatic ductal adenocarcinoma

39
Q

What are some causes of pancreatic ductal adenocarcinoma?

A
  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
Q

What is the difference between familial and hereditary pancreatic cancer?

A

Familial - pancreatic cancer in 2 or more first degree relatives
Hereditary - due to an identified causative underlying germline mutation

41
Q

Which hereditary cancer syndromes can cause pancreatic cancer?

A
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
Q

What is PanIN?

A

Pancreatic intra epithelial neoplasia

PanIN (1,2,3)

43
Q

Where are 70% of pancreatic cancers located?

A

head of pancreas

44
Q

What is a desmoplastic response?

A

intense non-neoplastic reaction by host to presence of tumour
forming firm fibrous tissue surrounding the tumour

45
Q

why does pancreatic cancer have such a high mortality?

A
  • early cancer is silent
  • present for at least a decade before detected
  • non specific symptoms - epigastric pain, radiating to back
46
Q

What is Trousseau’s syndrome?

A

migratory thrombophlebitis - poor prognostic factor of pancreatic carcinoma

47
Q

What is Courvoisier’s sign?

A

palpable gall bladder without pain - poor prognostic factor of pancreatic carcinoma

48
Q

What is a pancreatic neuroendocrine tumour?

A
  • 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
Q

What are the risk factors for pancreatic neuroendocrine tumours?

A

M=F
Risk factors - smoking, family history of cancer, alcohol, obesity, diabetes
-increased risk in MEN-1, NF-1, VHL

50
Q

What are some examples of neuroendocrine neoplasms and what cell type and clinical findings are associated to each?

A
  1. Insulinoma
    B cell
    Clinical finding - hypoglycaemia
  2. Glucagonoma
    A cell
  3. Somatostatinoma
    D cell
    clinical finding: hypochlorhydria

some can be non-functioning