Week 7/8 - D - Chronic pancreatitis, multiple endocrine neoplasia and pancreatic cancer

Question 1

Define chronic pancreatitis?

Answer 1

Chronic pancreatitis is a chronic, irreversible, inflammation and/or fibrosis of the pancreas, often characterized by severe pain and progressive endocrine and exocrine insufficiency.

Question 2

What is the main cause of chronic pancreatitis? What are other causes?

Answer 2

The main cause of chronic pancreatitis is due to excessive alcohol consumption Other causes include * Idiopathic * Smoking * Autoimmune Rarely - * Pancreatic duct obstruction eg stones, stricture, tumour * Cystic fibrosis

Question 3

How do patient with chronic pancreatitis tend to present? * What is the pain relieved by?

Answer 3

Presentation is similar to acute pancreatitis * Epigastric pain radiating through to the back that is relieved by sitting forward / hot water bottles * Pain typically worse following a meal * Steatorrhoea - sign of pancreatic insufficiency * Weight loss * Bloating, nausea, vomiting * Jaundice amy also be present

Question 4

What is the sign that may be seen on the patients abdomen due to chronic hot water bottle use? (can be seen in chronic hot water bottle use eg for people who sleep with it in between their legs to keep warm)

Answer 4

This is known as erythema ab igne - mottled dusky greyness on abdomen

Question 5

How is chronic pancreatitis diagnosed? What may be seen on AXR in a patient with chronic pancreatitis?

Answer 5

CHonic pancreatitis tends to be diagnosed using AXR may show speckled pancreatic calcification in 30% CT scan - pancreatitic calcifications confirm the diagnosis

Question 6

What is the protease synthesised by pancreatic acinar cells and secreted into the duodenum. It is not degraded during transit and is concentrated in the faeces? The levels of this enzyme are usually lowered in chronic pancreatitis due to progressive exocrine and endocrine insufficiency

Answer 6

Can measure faecal elastase levels * An indirect pancreatic function test; reduced in severe disease to

Question 7

What is the management of chronic pancreatitis?

Answer 7

STOP DRINKING AND SMOKING Manage any acute episodes appropriately (IV fluids and analgesia, ERCP + cholecystectomy if gallstones found) Replacement of pancreatic enzymes eg creon (pancrelipase)

Question 8

When is surgery carried out for chronic pancreatitis? What are the two types of surgery carried out?

Answer 8

Surgery is considered for patient with unremitting pain and severely decreased weight Options * Resectional surgery where pancreatectomy is carried out * or * Pancreaticjejunosotomy (pancreatic duct draining pocedure where jejunum is connected to pancreas)

Question 9

The two types of pancreaticojejunostomy carried out are known as Pustow procedure and Frey procedure What happens in these procedures?

Answer 9

Pustow procedure - involves a side-to-side anastomosis of the pancreatic duct and the jejunum Frey procedure - diseased portions of the pancreas head are cored out. pancraticojejunosotomy is then formed

Question 10

What are the complications of chronic pancreatitis?

Answer 10

Complications of chronic pancreatitis include: Chronic pain. Endocrine insufficiency with failure to produce insulin, causing and diabetes mellitus. Exocrine insufficiency with failure to produce digestive enzymes, causing maldigestion and malabsorption. Pancreatic calcification. Pseudocyst formation. Pancreatic cancer

Question 11

Pancreatic tumours What are the different tymours of the pancrease?

Answer 11

Exocrine pancreatic tumour - most common - adenocarcinoma Pancreatic neuroendocrine tumours - these can be benign or malignant- * gastrinomas, * insulinomas, * glucagonomas, * somtatostatinomas * VIPomas

Question 12

Pancreatic neuroendocrine tumours - these can be benign or malignant- * gastrinomas, * insulinomas, * glucagonomas, * somtatostatinomas * VIPomas What are the associated features of each of the pancreatic tumours listed above? (We will ask about the symptoms of pancreatic tumours in general later)

Answer 12

* Gastrinomas - gastrin secreting tumours -> HCl secretion leading to duodenal/stomach peptic ulcers * Insulinomas - insulin secreting tumours, encourages glucose uptake causing severe hypoglycaemia * Glucagonomas - glucagon secreting tumours -> hyperglycaemia, diabets * somatostatinomas - cause diabeets mellitus and steatorrhoea * VIPomas - severe diarrhoea, hypokalaemia, achlorydia

Question 13

Pancreatic endocrine tumours can occur * Sporadically or * Are associated with a condition where there are functioning hormone-producing tumours in multiple organs What is this condition known as? What is the inheritance?

Answer 13

Known as multiple endocrine neoplasia - it has an autosomal dominant inheritance

Question 14

There are 3 different types of multiple endocrine neoplasia MEN 1 MEN 2a MEN 2b What are the gene mutations in each?

Answer 14

MEN 1 * MEN1 gene mutation (tumour suppressor gene) MEN2a and MEN2b * RET gene mutation (proto-oncogene)

Question 15

There are 3 different types of multiple endocrine neoplasia MEN 1 MEN 2a MEN 2b * Which type is associated with pancreatic endocrine tumours? * What other features are associated with this type? * What is the mutation in this type?

Answer 15

MEN 1 (3Ps) * Parathyroid - hyperparathyroidism due to parathyroid hyperplasia * Pancreas endocrine tumours (insulinoma, gastrinoma, glucagonoma) * Pituitary tumours - prolactinoma or GH tumou

Question 16

What are the tumours associated with MEN2a and MEN2b?

Answer 16

MEN2a (1M, 2Ps)- Medullary thyroid cancer Parathyorid - hyperparathyroidism due to parathyroid hyperplasia Phaechromcoytoma - usually bilateral MEN2b (2Ms, 1P) Medullary thyroid cancer Marfinoid status / mucosal neuromas Phaechomocytoma

Question 17

Pancreatic cancer The most common type of pancreatic tumour is the adenocarcinoma of the pancreas (tumour of the exocrine cells) Where in the pancreas does this tumour tend to occur?

Answer 17

Majority of pancreatic adenocarcinoma arise in * Head of pancreas * Followed by body of pancreas * Followed by tail of pancreas A few arise in the ampulla of vater (ampullary tumour)

Question 18

What are the risk factors for pancreatic cancer?

Answer 18

Risk factors increasing age smoking High fat and red or processed meat diet alcohol diabetes chronic pancreatitis multiple endocrine neoplasia

Question 19

Is the prognosis of pancreatic cancer good? What are the symptoms of pancreatic cancer?

Answer 19

Pancreatic cancer is often diagnosed late as it tends to present in a non-specific way and therefore has a poor prognosis Presentation typically * Painless obstructive jaundice * Loose, pale stools * Dark urine * Weight loss * Back pain

Question 20

What is seen on examination in a patient with pancreatic cancer? * What is courvoisier’s law?

Answer 20

* Jaundice + palpable gallbladder * Epigastric mass * HEpatosplenomegaly Courvoisier’s law states that in the presence of painless obstructive jaundice, a palpable gallbladder is unlikely to be due to gallstones (if seen with painless jaundice, the cause is not gallstones)

Question 21

Migratory thrombophlebitis is an acquired blood clotting disorder that results in inflammation of a vein due to a blood clot * Although not always associated with an internal malignancy, many cases do show an underlying cancer. What is this syndrome known as? What happens in migratory thrombophlebitis? What cancers are associated with it?

Answer 21

Trousseau’s syndrome -> vessel inflammation due to blod clot (thrombophlebitis) which are recurrent or appearing in different locations over time eg arm vein becomes swollen/red/ tender then leg vein (thrombophlebitis migrans Particularly associated with pancreatic, gastric and lung cancer

Question 22

What is seen on the bloods in pancreatic cancer? How is pancreatic cancer diagnosed?

Answer 22

Bloods show a cholestatic jaundice * Ultraosund scan is the intiial investifation * Tripe phase CT scan is the diagnostic test of chocie MRI/MRCP can help aid the diagnsosis/localise the site of obstruction

Question 23

Majority of pancreatic cancers are inoperable at presentation How are inoperable cases managed intitially?

Answer 23

Initial inoperable management * ERCP (or PTC - Percutaneous Transhepatic Cholangiogram) - either can be used for stent insertion into * Palliative decompression of obstructed biliary ducts

Question 24

In operable cases of pancreatic cancer, surgery is carried out What are the different surgical procedures?

Answer 24

Surgery * Pancreaticoduodenecomy (Whipple’s procedure) is carried out for head of the pancreas cancers * Distal pancreatectomy for tail of the pancreas cancer * Total pancreatectomy

Question 25

What is involved in Whipple’s procedure?

Answer 25

Pancreaticoduodenoectomy * Head of pancreas and duodenum are removed * Choledochojejunostomy (hepatoduodenostomy) is formed * Duodenojejunosotomy is formed * Pancreaticojejunostomy is formed

Question 26

What else is given to patients who undergo surgery to treat the pancreatic cancer?

Answer 26

Adjuvant chemotherapy is usually given following surgery (sometimes also adjuvant readiotherapy)