Theme 11 L3,4: Liver tumours, biliary and pancreatic pathology Flashcards
What is an adenoma of the liver?
-a benign proliferation of epithelial cells (hepatocytes)
What are adenomas of liver cells often driven by?
exogenous steroids e.g OCP, anabolic steroids
If an adenoma ruptures, what complication could arise?
Haemoperitoneum
What condition can cause tiny white nodules on the liver?
adenoma of bile ducts
What is a von meyengerg complex?
multiple bile duct hamartomas or biliary microhamartomas
What are the two benign tumours of blood vessels of the liver?
- Hepatic haemangioma
- Focal nodular hyperplasia
- young females
- a regenerative, arterialised nodule
What is the most common malignant liver tumour?
hepatocellular carcinoma
What is the aetiology behind hepatocellular carcinoma?
- cirrhosis
- hepB/C
- fatty liver disease or alcohol
What is secreted by malignant liver cells which can be detected by the blood and measured?
AFP (alpha feroprotein)
What is a cholangiocarcinoma?
malignant tumour of the bile duct cells
What are some causes of cholangiocarcinoma?
chronic inflammation - P.S.C, liver fluke
What is the main clinical feature of cholangiocarcinoma?
jaundice
What is an angiosarcoma?
- aggressive malignant tumour of blood vessel
- strongly associated with toxins e.g vinyl chloride, thorotrast
Majority of liver tumours are a result of metastasis. What are the common sites of origin?
lung, breast, colon, pancreas
What is the aetiology of gall stones?
- cholesterol, bile salts, bacterial growth + calcification
- slowly form a stone (calculus)
What are the risk factors for gallstones?
female
middle aged
over weight
What are the clinical features of gall stones?
crampy pain
- 80% asymptomatic
What are the complications of gallstones?
- obstruction at neck - pain; obstruction
- chronic cholecysitis
- perforation
- obstruction at pancreatic level - pancreatitis
What is chronic cholecystitis?
- inflammation of the gall bladder
- due to chemical or bacterial causes
- over time causes fibrosis, ulceration of the gall bladder
For chronic cholecystitis:
- Clinical features?
- Diagnosis?
- Treatment?
- Pain (RUQ), fever, jaundice
- USS
- Cholecystectomy
How much of the pancreas is exocrine?
85%
What do exocrine glands of the pancreas secrete?
digestive enzymes - trypsin, lipase, phospholipase, elastase, amylase which require activation within the duodenum
Which digestive enzyme of the pancreas is a marker of pancreatic inflammation?
amylase
What is the endocrine function of the pancreas?
islets of langerhans secrete peptide hormones into the blood e.g insulin and glucagon
What is pancreatitis associated with?
injury to the exocrine parenchyma
What are the causes of acute pancreatitis?
-gallstones (50%)
-alcohol (25%)
rarer causes - vascular insufficiency, viral infections, hypercalcaemia
-idiopathic
What is hereditary pancreatitis defined by?
- recurrent attacks of severe pancreatitis
- usually begins in childhood
Which two mutations are implicated in hereditary pancreatitis?
- PRSS1 inherited mutations - autosomal dominant (inability to activate trypsin)
- SPINK 1 Gene - autosomal recessive (autodigestion of pancreas)
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
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
What is:
- Grey turner’s sign
- Cullen’s sign
- Haemorrhage into the sub cutaenous tissues of flank
2. Peri umbilical haemorrhage
What are the complications of acute pancreatitis?
- shock
- intravascular coagulopathy
- haemorrhage
- pseudocysts (collections of pancreatic juice secondary to duct rupture)
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
What are the clinical features of chronic pancreatitis?
- intermittent abdo pain, back pain and weight loss
- malnutrition
- diabetes
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
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
What is seen in patients with chronic pancreatitis associated with excess alcohol?
calculi
What is the most common type of pancreatic cancer?
pancreatic ductal adenocarcinoma
What are some causes of pancreatic ductal adenocarcinoma?
- cigarette smoking
- family history
- nutritional and dietary factors rich in red meats, obesity, low physical activity, low intake of fruit and veg
- other conditions - chronic pancreatitis, DM
- alcohol
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
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
What is PanIN?
Pancreatic intra epithelial neoplasia
PanIN (1,2,3)
Where are 70% of pancreatic cancers located?
head of pancreas
What is a desmoplastic response?
intense non-neoplastic reaction by host to presence of tumour
forming firm fibrous tissue surrounding the tumour
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
What is Trousseau’s syndrome?
migratory thrombophlebitis - poor prognostic factor of pancreatic carcinoma
What is Courvoisier’s sign?
palpable gall bladder without pain - poor prognostic factor of pancreatic carcinoma
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
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
What are some examples of neuroendocrine neoplasms and what cell type and clinical findings are associated to each?
- Insulinoma
B cell
Clinical finding - hypoglycaemia - Glucagonoma
A cell - Somatostatinoma
D cell
clinical finding: hypochlorhydria
some can be non-functioning
