PANCREATIC PATHOLOGY Flashcards
Exocrine
most of pancreas (85%). glands- ducts-duodenum. Secrete digestive enzymes -trypsin, lipase, phospholipase, elastase, amylase (marker of pancreatic inflammation) which require activation.
Endocrine
Islets of Langerhans. Secrete peptide hormones into blood (e.g. insulin and glucagon). This is a small component and only occupies 1-2% of pancreas.
Acute and chronic pancreatitis
Inflammation (itis) of pancreas.
Largely caused by gallstones and alcohol. Definition: Acute = reversible. Chronic = irreversible. A
ACUTE pancreatitis
ACUTE: mainly affects the EXOCRINE FUNCTION Epidemiology & Aetiology: Relatively 10-20 per 100,000 in western countries. Gallstones (50% cases and more common in women) Alcohol (25% cases are more common in men).
Hereditary pancreatitis –> recurrent attacks of severe pancreatitis. Usually beings in childhood: PRSS1 inherited mutations –AD. SPINK1 gene (AR) Pathogenesis: Varies according to aetiology. 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 – haemorrhage into the subcutaneous tissues of flank, Cullen’s sign – per umbilicus). Hypocalcaemia (fatty acids bind caions), hyperglycaemia, abscess formation, pseudocysts
Key clinical features of acute pancreatitis
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 canhave high mortality.
Raised serum amylase/lipase (>3x normal) due to digestive enzymes being released.
Persistent hypocalcaemia is a poor prognostic sign. Complications: Shock, intravascular coagulopathy, haemorrhage into pancreas and pesuodcysts.
Distorted ducts. Calculi, esp in alcohol induced.
abdo & back pain, weight loss
Chronic pancreatits
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 (late feature). Leads to malnutrition and diabetes.
Fibrosis of exocrine tissue – can mimic carcinoma macroscopically and microscopically
Epidemiology: 26/100,000 population
Localised, irregular involvement of the gland early on, later global atrophy. Dilated and Toxic –alcohol, cigarette smoke, drugs, hypercalcemia, hyperparathyroidism infections. Idiopathic. Genetic CFTR (cystic fibrosis gene), PRSS1, SPINK 1 mutations. Autoimmune. Recurrent acute pancreatitis. Obstruction of main duct – cancer.
Mortality rate - nearly 50% with 20-25 years of disease onset.
Pancreatic adenocarcinoma (primary pancreatic cancer) Arises from endocrine potion
3% of all cancers (10th most common)
Most common type of pancreatic cancer (up to 90% of all pancreatic neoplasms).
5 year survival 4%. 60-80 years, rare before 40 years. 1.3M:1F. Complex and multifactorial.
Cigarette smoking (increases risk 2-3 times) and family history in 10% cases. Family history increases your risk by 9 times. HNPCC, BRACA 2 increased risk.
60-70% cases are found in the head of the pancreas.
Present for at least a decade (10 years) before detected. Early cancer is silent. Mean survival untreated is 3-5 months. 10-20 months post-surgery but only 10-20% are resectable at the time of diagnosis. Non-specific symptoms -epigastric pain, radiating to back, Weight loss, painless jaundice, pruritus and nausea. Trousseau’s syndrome (migratory thrombophlebitis) and Courvoisier’s sign (palpable gallbladder without pain).
Poor prognostic factors: extension of tumor outside the pancreas and spread to lymph nodes. Tumor in the body and the tail can be small but inoperable. You don’t get jaundice in these locations
Pancreatic neuroendocrine tumours
Arises from endocrine portion
Derived from islet cells. Present with a wide range of clinical symptoms – depending upon their endocrine effects.
Spectrum of behaviour – from benign to malignant Epidemiology: Uncommon < 3% pancreatic neoplasms. Incidence <1 per 100,000 per year. Autopsies show higher incidence, so people live with them undetected. Increased risk MEN 1 Aetiology: Pathogenesis: Tumours are usually well circumscribed, sometimes encapsulated, and solid.
Between 10-50mm. Occur anywhere in pancreas. Can be multiple
Well-differentiated neuroendocrine tumours
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
Poorly differentiated neuroendocrine carcinomas
< 1% of all pancreatic tumour- rare. 2-3% of all pancreatic endocrine tumours. Males > Females. 40-75 years. Advanced disease at presentation. Prognosis without treatment 1-2 months, with chemotherapy-to 50 months
