Parcreatic pathology

Question 1

What are the exocrine functions of the pancreas?

Answer 1

Most of pancreas (85%)

Glands- ducts-duodenum

Secrete digestive enzymes

• trypsin
• lipase
• phospholipase
• elastase
• amylase (marker of pancreatic inflammation) which require activation

Question 2

What are the endocrine functions of the pancreas?

Answer 2

Islets of Langerhans

Secrete peptide hormones into blood (e.g. insulin and glucagon)

Question 3

What are the clinical features of acute pancreatitis?

Answer 3

Usually presents as an emergency requiring admission to hospital

Sudden onset of severe abdo pain radiating to back

Nausea and vomiting

May be mild (recovery within 5-7 days) but can be serious with high mortality

Raised serum amylase/lipase (>3x normal)

Question 4

What are the possible aetiologies of acute pancreatitis?

Answer 4

Gallstones (50% cases)

Alcohol (25% cases)

Rare causes (

Question 5

What is the pathogenesis of acute pancreatitis?

Answer 5

Leakage and activation of pancreatic enzymes

Amylase released into blood

Mild pancreatitis
- swollen gland with fat necrosis

Severe

• swollen
• necrotic gland with fat necrosis and haemorrhage
• Grey Turner’s sign
• Cullen’s sign

Hypocalcaemia (fatty acids bind calcium ions), hyperglycaemia, abscess formation, pseudocysts

Question 6

What are the features of fat necrosis?

Answer 6

Chalky white material containing calcium salts which have been freed up by lipase mediated cleavage of fatty acids.

Question 7

What is Grey Turner’s sign?

Answer 7

Haemorrhage into the subcutaneous tissues of flank. Seen in severe acute pancreatitis.

Question 8

What is Cullen’s sign?

Answer 8

Superficial edema and bruising in the subcutaneous fatty tissue around the umbilicus. Seen in acute pancreatits.

Also in ruptured ectopic pregnancy.

Question 9

What are the complications of acute pancreatitis?

Answer 9

Shock

Intravascular coagulopathy

Haemorrhage

Pseudocysts (collections of pancreatic juice secondary to duct rupture)

Question 10

What is chronic pancreatitis?

Answer 10

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

Leads to malnutrition and diabetes

Question 11

What is the overlap between acute and chronic pancreatitis?

Answer 11

Recurrent acute can develop chronic pancreatitis

Overlap causative factors

Both genetic and environmental

Experimental protocols can be modified to induce each condition

Question 12

What are the causes of chronic pancreatitis?

Answer 12

Toxic

• alcohol
• cigarette smoke
• drugs
• hypercalcaemia
• hyperparatyroidism infections

Genetic
- CFTR
- PRSS1
- SPINK 1 mutations
Obstruction of  main duct 
- cancer
- scarring

Recurrent acute pancreatitis

Autoimmune

Idiopathic

Question 13

Excluding CF, what percentages of chronic pancreatitis is caused by alcohol consumption.

Answer 13

90-95%

Question 14

What percentage of alcoholics develop pancreatitis?

Answer 14

10%

Question 15

What is a strong independent risk factor for pancreatitis?

Answer 15

smoking

Question 16

What are the complications of chronic pancreatitis?

Answer 16

Malabsorption of fat (lack of lipases)

• Steatorrhoea
• Impairment of fat soluble vit absorption –A,D, E and K
• Diarrhoea, weight loss and cachexia

Diabetes (late feature)

Pseudocysts

Stenosis of common bile duct/duodenum

Mortality rate - nearly 50% with 20-25years of disease onset

Question 17

What are the gross and microscopic features of pancreatitis?

Answer 17

Localised, irregular involvement of the gland early on, later global atrophy.

Dilated and distorted ducts

Calculi, esp in alcohol induced

Fatty replacement

Pseudocyst formation

Question 18

What is the most common type of pancreatic cancer?

Answer 18

Ductal adenocarcinoma

Up to 90%

Question 19

What is the 5-year-survival rate for pancreatic adenocarcinoma?

Answer 19

4%

Question 20

What is the typical age range for for pancreatic adenocarcinoma?

Answer 20

60-80 years

Question 21

How much does smoking increase the risk of developing pancreatic adenocarcinoma?

Answer 21

2-3x

Drops with abstinence

Question 22

What are the other risk factors for pancreatic adenocarcinoma (other than smoking).

Answer 22

Heavy alcohol intake

Diet rich in red meats

Obesity

Hereditary (10%)

Chronic pancreatitis

Question 23

What is the distribution of pancreatic adenocarcinoma about the pancreas?

Answer 23

60-70% head of pancreas

5-15% body

10-15% tail

5-15% diffuse involvement

Question 24

For how long is pancreatic adenocarcinoma present before it’s detected?

Answer 24

At least a decade

Question 25

What is Trousseau’s syndrome?

Answer 25

Migratory thrombophlebitis

Question 26

What is Courvoisier’s sign?

Answer 26

Palpable gallbladder without pain

Question 27

What are the symptoms of pancreatic cancer?

Answer 27

Non-specific

Epigastric pain, radiating to back

Weight loss, painless jaundice, pruritis and nausea.

Trousseau’s syndrome and Courvoisier’s sign (palpable gallbladder without pain)

Distant metastases (liver, peritoneum, lung)

Diabetes – increases risk and can be a presenting complication

Question 28

What is the prognosis for pancreatic adenocarcinoma?

Answer 28

Almost universally fatal (incidence nearly equals mortality)

Mean survival untreated is 3-5 months

10-20 months post- surgery but only 10-20% are resectable at the time of diagnosis.

Question 29

What are the poor prognostic factors for pancreatic adenocarcinoma?

Answer 29

Extension of tumour outside the pancreas

Metastatic spread to local lymph nodes

Vascular and perineural invasion

Grade of differentiation

Question 30

What hereditary cancer syndromes cause 5-10% of pancreatic adenocarcinoma?

Answer 30

HNPCC
- DNA mismatch repair

FAMMM
- p16/cdkn2A

Familial breast cancer
- BRCA2

Ataxia-telangiectasis
- ATM

Von Hippel-Lindau
- VHL

Familial pancreatitis
Cationic trypsinogen, - SPINK1

Peutz-Jeghers
- LKB1/STK11

Question 31

How common are pancreatic neuroendocrine tumours?

Question 32

What is the age range for pancreatic neuroendocrine tumours?

Answer 32

20-60 years

Question 33

What conditions increase your risk of pancreatic neuroendocrine tumours?

Answer 33

MEN1

von Hippel Lindau

Question 34

What are the features of well-differentiated neuroendocrine tumours?

Answer 34

Uncommon 1-2% all pancreatic neoplasms

Any age, rare in children

7-13% multiple (MEN-1)

15-35% non-functioning

Single tumours often produce multiple hormones but usually single hyperfunctional syndrome

Question 35

What are the features of poorly differentiated neuroendocrine carcinomas?

Answer 35

Rare

Females

40-75 years

Advanced disease at presentation

Prognosis without treatment 1-2 months, with chemotherapy up to 50 months

Question 36

What are the macroscopic features of pancreatic neuroendocrine tumours?

Answer 36

Usually well circumscribed, sometimes encapsulated

Solid

10 - >50mm

Occur anywhere in pancreas

Can be multiple