Coupon Accepted Successfully!



Embryological development
  1. The pancreas develops from separate ventral & dorsal buds that arise from the junction of primitive foregut & midgut.
  2. The dorsal bud enlarges towards the left, and forms the main bulk of the adult gland.
  3. The ventral bud, which is closely associated with the developing common bile duct is brought into apposition with the dorsal system only in the seventh week of intrauterine growth, following its rotation.
  4. Both parts of the primitive pancreas contain axial ducts, the dorsal duct arising from the duodenal wall, and the ventral duct from the common bile duct. When they fuse, the ventral duct (of Wirsung) becomes continuous with the dorsal duct (of Santorini) to form the main pancreatic duct.
  5. The common bile duct and pancreatic ducts therefore enter the duodenum at the main papilla, while the portion of the dorsal duct within the head of the pancreas enters the duodenum proximally to the main papilla, through a small accessory, or minor papilla.
  6. Complete failure of the two duct systems to fuse results in a pancreas divisum (Fig 2- 5%) and may predispose to pancreatitis.
  7. Failure of the body of the ventral bud to rotate may give rise to an annular pancreas surrounding the second part of the duodenum.
  8. This may be responsible for duodenal obstruction.
Annular pancreas:
  1. A congenital anomaly characterized by a ring of normal pancreatic tissue encircling and sometimes obstructing the descending part of the duodenum.
  2. The annulus represents the ventral part of the pancreas that remains fixed to the duodenum. In the extramural type, the annulus is drained by ducts running around the duodenum to join the main pancreatic duct.
  3. Associated features with Annular pancreas are: Absent spleen, Duodenal stenosis, Ectopic spleen, Polysplenia, Situs inversus-abdominal, Small bowel atresia/ obstruction.
Acute Pancreatitis
  1. Acute pancreatitis is an inflammatory process in which pancreatic enzymes autodigest the gland.
  2. The gland can heal without any impairment of function or any morphologic changes.
  3. It can recur intermittently, contributing to the functional and morphologic loss of the gland. Recurrent attacks are referred to as chronic pancreatitis.
  1. Pathophysiology:
    1. Since the pancreas is located in the retroperitoneal space with no capsule, inflammation can easily spread.
    2. In acute pancreatitis, parenchymal edema and peripancreatic fat necrosis occur first, called acute edematouspancreatitis.
    3. When necrosis involves the parenchyma, accompanied by hemorrhage and dysfunction of the gland, the inflammation evolves into hemorrhagic or necrotizing pancreatitis.
    4. Pseudocysts and pancreatic abscesses can result from necrotizing pancreatitis, due to enzymes being walled off by granulation tissue (pseudocyst formation) or bacterial seeding of pancreatic or peripancreatic tissue (pancreatic abscess formation).
    5. Fat necrosis causes hypocalcemia. Pancreatic B cell injury may lead to hyperglycemia.
    6. Trypsin and chymotrypsin are the initiating enzymes; their release can in turn result in the release and activation of other proenzymes (including proelastase, procollagenase and phospholipases).
    7. When the pancreas is inflamed but remains viable, the condition is termed interstitial pancreatitis; this may occur in up to 80% of cases.
    8. In the remaining cases, there is significant pancreatic necrosis resulting from disruption of the microcirculation, destruction of the pancreatic parenchyma and peripancreatic
    9. All episodes of acute pancreatitis begin with severe pain, generally following a substantial meal.
    10. The pain is usually epigastric, but can occur anywhere in the abdomen or lower chest. It has been described as "knifing" or "boring through" to the back, and may be relieved by the patient leaning forward.
    11. It precedes the onset of nausea and vomiting, with retching often continuing after the stomach has emptied.
    12. Vomiting does not relieve the pain, which is more intense in necrotizing than in edematous pancreatitis.
  1. Clinical feature:
    1. Epigastric pain or right upper quadrant pain radiating to the back is the hallmark of pancreatis
    2. Nausea/vomiting
    3. Fever
  2. Physical:
    1. Tachycardia/ Tachypnea/ Hypotension/ Fever
    2. Abdominal tenderness, distension, guarding and rigidity. In severe cases, there may be a Grey Turner sign (i.e., bluish discoloration of the flanks) and Cullen's sign (i.e., bluish discoloration of the periumbilical area) due to the retroperitoneal leak of blood from the pancreas in hemorrhagic pancreatitis.
    3. Mild jaundice
    4. Diminished or absent bowel sounds
    5. Basal rales in lungs, (especially in the left due to contiguous spread of inflammation).
    6. In the extremities, muscular spasm may, be noted secondary to hypocalcemia.
    7. Multisystem organ failure (ARDS, renal failure from ATN), shock, DIC and hemorrhage.
    8. Pleural effusions, pneumonia, and atelectasis.
    9. Formation of pancreatic fluid collections (pseudocysts and abscesses) account for 70% to 80% of mortality.
    10. Ileus, colonic obstruction, CNS hypoperfusion with confusion, etc.
  3. Causes:
    1. The most common cause of acute pancreatitis is gall stone.
    2. Long-standing alcohol intake.
    3. Medications: azathioprine, corticosteroids, sulfonamides, thiazides, furosemides, NSAIDs, mercaptopurine, methyldopa, tetracyclines DDI (dideoxycytosine), DDC (dideoxyinosine), azathioprine, valproic acid, acetaminophen, and others.
    4. Endoscopic retrograde cholangiopancreatography (ERCP)
    5. Hypertriglyceridemia
    6. Peptic ulcer disease
    7. Abdominal or cardiopulmonary bypass surgery: insulting the gland by ischemia
    8. Trauma to the abdomen or to the back.
    9. Carcinoma of the pancreas, which may lead to pancreatic outflow obstruction
    10. Viral infections: cytomegalovirus, hepatitis virus, EBV, CMV and paramyxovirus [mumps], togavirus [rubella], cytomegalovirus, adenovirus, HIV, coxsackie B,
    11. Bacterial infections, such as mycoplasma, Campylobacter, Legionella, Mycobacterium tuberculosis, M. avium
    12. Intestinal parasites: ascaris, Opisthorchis [clonorchiasis], which blocks outflow
    13. Pancreas divisum
    14. Scorpion and snake bites
    15. Vascular factors, such as ischemia or vesculitis, Connective-tissue disorders (SLE, polyarteritis nodosa, sarcoidosis) 
Causes of Acute pancreatitis
Common causes Uncommon causes
• Gallstones including microlithiasis (MC)
• Alcohol (2nd MC)
• Hypertriglyceridemia
• Blunt abdominal trauma
• Postoperative
• Drugs
• Sphincter of Oddi dysfunction
• Vascular causes and vasculitis (ischemic- hypoperfusion states after cardiac surgery)
• Connective tissue disorders                    
• TTP                                                               
• CA pancreas                                               
• Hypercalcemia (Hyperparathyroidism)
• Periampullary diverticulum                     
• Pancreas divisum                                       
• Hereditary pancreatitis                                            
• Cystic fibrosis                                             
• Renal failure                                               
Rare Causes:
• Infections (CMV, Coxsackie, Mumps, echovirus, parasites)
• Autoimmune (Sjögren syndrome)
Drugs Associated With Pancreatitis
Definite Cause Probable Cause
• 5-Aminosalicylate
• 6-Mercaptopurine
• Azathioprine
• Cytosine arabinoside
• Dideoxyinosine
• Diuretics (Furosemide, Thiazide)
• Metronidazole
• Pentamidme
• Tetracycline
• Valproic acid
• Trimethoprim sulfamethoxazole
• Estrogens
• Acetaminophen
• Alpha-Methyl-dopa
• Isoniazid
• L-Asparaginase
• Phenformin
• Procainamide
• Sulindac
Objective measurements such as Ranson's criteria, show a good correlation with the risk of major complications and death.
TABLE. Ranson's criteria

Ranson developed a series of different criteria for the severity of acute pancreatitis.

Ranson's Prognostic Signs of Pancreatitis
Criteria for acute pancreatitis not due to gallstones 
At admission During the initial 48 h
Age >55 y Hematocrit fall >10 points
WBC>16,000/mm3 BUN elevation >5 mg/dL
Blood glucose >200 mg/dL Serum calcium <8 mg/dL
Serum LDH >350 IU/L Arterial PO2<60 mm Hg
Serum AST >250 U/dL Base deficit >4 mEq/L, Estimated fluid sequestration >6 L
Criteria for acute gallstone pancreatitis 
At admission During the initial 48 h
Age >70 y Hematocrit fall >10 points
WBC>18,000/mm3 BUN elevation >2 mg/Dl
Blood glucose >220 mg/dL Serum calcium <8 mg/dL
Serum LDH >400 IU/L Base deficit >5 mEq/L
Serum AST >250 U/dL Estimated fluid sequestration >4 L
Criteria During initial 48 Hours
· Age >55 years · Blood glucose >180 mg/dL
· WBC count >15,000 cell/mm · Serum LDH >600 IU/L
· Blood urea nitrogen >45 mg/dL · Serum calcium <8 mg/dL
· Arterial PO2 <60 mm Hg · Serum albumin <3.3 g/dL
The 12 physiologic variables are
· Temperature · Oxygenation · Hematocrit
· Heart rate · Arterial pH · WBC count
· Respiratory rate · Serum potassium · Glasgow Coma Scale
· Mean arterial blood pressure · Sodium
· Creatinine
  1. Lab Studies:
    1. Leukocytosis (WBC > 12000)
    2. Patient may have hypocalcemia caused by "soap" formation (saponification of triglycerides and calcium).
    3. Frequently have hypomagnesemia.
    4. Amylase (preferably the Amylase P): Levels more than 3 times the norm strongly suggest the diagnosis of  acute pancreatitis.
    5. Lipase is more sensitive and specific if symptoms have been present for more than 24 hours. Both amylase and lipase levels may be normal in a patient with CT-proved pancreatitis. Lipase remains high for 7-14 days
    6. Most sensitive or therapeutic value for pancreatitis is S. trypsin level.
    7. Liver function tests (alkaline phosphatase, SGPT, SGOT, G-GT) and bilirubin should be ordered, particularly with biliary origin Pancreatitis.
​​Extra Edge: Extrapancreatic conditions may cause hyperamylasemia
Abdominal conditions that may cause elevations in the serum amylase includes:
  1. Perforated peptic ulcer  
  2. Cholecystitis  
  3. Generalised peritonitis  
  4. Intestinal obstruction
  5. Mesenteric infarction  
  6. Ruptured AAA
  7. Ruptured ectopic pregnancy
  8. Two-thirds of diabetic ketoacidosis is associated with hyperamylasemia and may present a confusing clinical picture, particularly when it is associated with shock
Serum lipase also rises rapidly, within hours after an attack
  1. Reference range for serum lipase is ,130 U/l
  2. Lipase is measured via turbidimetric method:
  3. Sensitivity of lipase for acute pancreatitis approaches 100%
  4. Lipase lacks specificity and may be elevated in
    1. Acute cholecystitis        
    2. Choledocholithiasis    
    3. Mesenteric infarction                              
    4. ​Intestinal obstruction
Urine amylase
  1. Urine amylase rises a few hours after the rise sin serum amylase and lipase
  2. Urine amylase remains elevated for approximately seven to ten days
  3. The rise in urine amylase is secondary to an increase in the renal clearance of amylase
  4. Amylase clearance is used to differentiate between a patient with macroamylasemia secondary to hyperamylasemia from a patient with pancreatitis
  5. Normal ratio for amylase clearance is between 2-5%
  6. In macroamylasemia, the ratio is decreased
  7. In pancreatitis, the ratio is increased
  8. Amylase clearance= Urine amylase concentration x Serum creatinine concentration / Serum amylase concentration x Urine creatinine concentration
Imaging Studies: Plain KUB (Kidney/Ureter/Bladder):
  1. To exclude viscus perforation (air under the diaphragm).
  2. Chronic pancreatitis, peripancreatic calcifications may be noted.
  3. Radiography may reveal a "sentinel loop,'' a localized ileus in the midepigastric region. Pleural effusions may also be present.
  4. Ultrasound can be used as a screening test
  5. CT scan is gold standard in diagnostic imaging for acute pancreatitis.
  6. CT scan is the most reliable imaging modality in the diagnosis of acute pancreatitis. The criteria for the diagnosis are divided by Balthazar into 5 grades
    1. Grade A: Normal pancreas
    2. Grade B: Focal or diffuse gland enlargement
    3. Grade C: Intrinsic gland abnormality seen by haziness on the scan
    4. Grade D: Single ill-defined collection or phlegmon
    5. Grade E: Two or more ill-defined collections or the presence of gas in or nearby, the pancreas
  1. Treatment
    Emergency Department Care: 80% patients respond to conservative treatment.
Medical Treatment of Acute Pancreatitis
  • Analgesics (Buprenorphine, meperidine)
  • IV fluid and colloids
  • No oral alimentation
  • Treatment of hypocalcemia if symptomatic
  • Antibiotics
General Surgery should be consulted in the following cases:
  1. Absolute indication –      
    1. Gas bubbles on CT scan
    2. Positive bacterial culture or FNA.
  2. Relative indication –
    1. >50% necrosis
    2. Failure to improve despite medical therapy
    3. Sign of organ failure. 
Algorithm for managing acute pancreatitis (Ref: Schwartz's Principles of Surgery 9th Edition Ch 33)
  1. The most common cause of death in acute pancreatitis is secondary infection.
  1. The use of octreotide has not shown to improve outcome.
  2. Infected pancreatic necrosis is due to seeding of bacteria into the inflammation.
  3. An acute pseudocyst is an effusion of pancreatic juice that is walled off by granulation tissue after an episode of acute pancreatitis.
  4. Hemorrhage into the GI tract retroperitoneum or the peritoneal cavity is possible.
  5. Intestinal obstruction or necrosis
  6. ComCBD obstruction by a pancreatic abscess, pseudocyst or biliary stone.
  7. Internal pancreatic fistula from pancreatic duct disruption or a leaking pseudocyst
  1. Complications:-
Metabolic Hypocalcemia, hyperglycemia, hypertriglyceridemia, acidosis
Respiratory Hypoxemia, atelectasis, effusion, pneumonitis
   Acute respiratory distress syndrome (ARDS)
Renal Renal artery or vein thrombosis
Renal failure
Circulatory Arrhythmias
Hypovolemia and shock; myocardial infarct
Pericardial effusion, vascular thrombosis
Gastrointestinal Ileus
Gastrointestinal hemorrhage from stress ulceration; gastric varices (secondary to
splenic vein thrombosis)
Gastrointestinal obstruction

Portal vein thrombosis
Neurologic Psychosis or encephalopathy (confusion, delusion and coma)
Cerebral emboli, Blindness (angiopathic retinopathy with hemorrhage)
Hematologic Anemia, DIC (disseminated intravascular coagulopathy)
Dermatologic Painful subcutaneous fat necrosis

Chronic Pancreatitis
  1. Chronic pancreatitis is defined as a continued inflammation characterized by irreversible morphologic changes.
  2. These changes include fibrosis, ductal abnormality, calcification and cellular atrophy.
  3. Causes
    1. Alcohol is the major etiologic factor, accounting for about 75% of the cases.
    2. Repeated attacks of gallstone-related pancreatitis
    3. Diabetes
    4. Protein-calorie malnutrition,
    5. Hereditary pancreatitis,
    6. Cystic fibrosis
    7. Idiopathic causes.
  4. Classification
    1. Chronic pancreatitis is divided into two clinical types:
      1. Chronic pancreatitis
      2. Chronic relapsing pancreatitis
    2. In both, regardless of the cause, the gland is permanently damaged, morpho-logically and functionally causing recurrent painful attacks resembling acute pancreatitis.
  5. Pathology
    1. A cardinal feature of chronic pancreatitis is the presence of protein plugs and calcifications
    2. Proteinaceous material precipitates in the ducts and ductules, initially consisting mainly of pancreatic enzyme protein and a glycoprotein
    3. In late stages, calcium carbonate is added to the precipitates, giving rise to stones (pancreatic calculi)
    4. Protein plugs and calculi are rare in chronic obstructive pancreatitis
    5. Chronic obstructive pancreatitis is the result of occlusion of main pancreatic duct
    6. Any obstructing lesion--a tumor, a scar resulting from trauma, papillary inflammation, a congenital structure--is a potential cause
    7. Fibrosis is accompanied by uniform acinar atrophy
    8. Exocrine insufficiency ensues, but it's partially reversible if the obstruction is removed
  6. Clinical Manifestations
    1. Abdominal pain,
    2. Maldigestion,
    3. Diabetes (loss of exocrine and endocrine pancreatic function).
      1. The pain is localized to the upper abdomen, with radiation to subcostal regions and to the back.
      2. The pain is aggravated by meals and improves with fasting.
      3. When more than 90% of exocrine pancreatic function is lost, maldigestion and malabsorption ensue.
      4. This is manifested by steatorrhea (fat malabsorption) associated with diarrhea and bloating, azotorrhea    (protein malabsorption) and progressive weight loss.
      5. Latent fat-soluble vitamin deficiency (vitamins A, D, E and K) in addition to deficiencies of magnesium, calcium and essential fatty acids may occur and are closely related to dysfunction of fat digestion. Endocrine insufficiency presenting as diabetes mellitus may present at the same time as exocrine insufficiency or a few years later.
      6. Pancreatic pseudocyst is localized fluid collection occurring within a pancreatic mass or in the peripancreatic spaces following acute or chronic pancreatitis.
      7. Pancreatic ascites: Pancreatic ascites results from the leakage of pancreatic juices into the peritoneal cavity through a fistula or ruptured pseudocyst.
      8. It presents with gradually increasing massive ascites, high levels of amylase, abdominal pain and weight loss.
      9. Painful areas of subcutaneous fat necrosis result from the high levels pancreatic lipase.
      10. Common bile duct stricture: Common bile duct compression is another manifestation of chronic pancreatitis.
      11. As the distal common bile duct traverses the head of the pancreas, it may be narrowed secondary to inflammation, with edema or fibrosis of the gland.
      12. Some patients with chronic pancreatitis can be shown to have major ducts that have the appearance of a “chain of lakes” or a “string of pearls” that is the result of segments of dilated duct separated by areas of ductal stricture.
  1. Pseudocyst                                                  
  2. Common Bile Duct Obstruction
  3. Pancreatic Ascites                                     
  4. Pancreatic Pleural Effusion                         
  5. Splanchnic Venous Obstruction
  1. ​Diagnostic And Radiographic Evaluation
    1. Radiological evidence such as calcification seen exclusively in the ductal system on plain radiographic abdominal films, by ultrasonography or on computerized tomography suggests chronic pancreatitis.
    2. Abnormalities of the ducts associated with chronic pancreatitis can also be demonstrated by ERCP. In +-severe disease there is narrowing and dilation of ducts, stenosis and filling of side ductules.
    3. The only tests that appear to accurately measure pancreatic function in chronic pancreatitis are the direct tube tests that measure the response of the pancreas to various stimuli. The commonest manifestation is a decreased bicarbonate concentration (<50 mEq/L) and decreased volume of secretion.
Cambridge Criteria for Chronic Pancreatitis on ERCP
Stage Typical Changes
Normal Normal appearance of side branches and main pancreatic duct
Equivocal Dilatation/obstruction of < 3 side branches; normal main pancreatic duct
Mild Dilatation/obstruction of > 3 side branches; normal main pancreatic duct
Moderate Additional stenosis and dilatation of main pancreatic duct
Severe Additional obstructions, cysts, stenosis of main pancreatic duct; calculi
  1. Tests of pancreatic function
    1. Measurement of pancreatic exocrine function: The severity of maldigestion, its contribution to weight loss, & efficacy of pancreatic enzymes can be assessed
    2. Pancreatic function may be inferred by direct measurement of the components of pancreatic secretion
    3. Enzyme activity may be estimated by the ability of the pancreas to cleave a given substance
    4. Pancreatic integrity may be reflected by the level of pancreatic enzymes or hormones secreted in the bloodstream or by recovering enzymes from the stool
    5. Pancreatic dysfunction can be appreciated by the amount of undigested nutrients recovered in the stool
      1. Invasive Tests       
        • The Secretin Test        
        • The Lundh Test
      2. Noninvasive Tests
        • The NBT-PABA Test (Bentiromide Test)
        • Fecal Fat
        • Gold standard functional test for diagnosis is secretin stimulation tests.
        • Most sensitive test in early stages of disease is impaired bicarbonate production, on secretin  stimulation.
  2. Treatment
    Pain management:
    1. Abstinence from alcohol may decrease the frequency and severity of painful attacks.
    2. Large meals with foods rich in fat should be avoided.
    3. Analgesics should be given prior to meals.
    4. Large doses of pancreatic extracts may reduce the frequency and severity of the pain
    5. When there is a dilated pancreatic duct with obstructive areas, longitudinal pancreatojejunostomy (modified Pustow operation) may relieve pain.
    6. An alternative to surgical drainage may be achieved by endoscopic insertion of an an endoprosthesis (stent) into the pancreatic duct.
    7. Octreotide, a long-acting somatostatin analogue, decrease the pain of chronic pancreatitis.
    8. Denervation: Denervation procedures aim at interrupting the transmission of pain from the pancreas through the sympathetic nerve fibers
    9. Since the majority of sensory nerves to the pancreas transverse the celiac ganglia and splanchnic nerves, both transthoracic splanchnicectomy and ganglionectomy have been used.They offer variable degrees of pain relief
  3. Endoscopic Therapy
    Considerable progress has been achieved in applying endoscopic techniques to the management of chronic pancreatitis
    1. It is possible to remove stones in the pancreatic duct, directly by use of a basket
    2. Stents may be placed in strictured areas of the pancreatic duct.
    3. In cases of chronic pancreatitis associated with pancreas divisum, endoscopic sphincterotomy of the minor papilla and stent placement across the papilla are successful in decreasing the frequency and severity of the attacks
  4. Malabsorption:
    1. Administration of high-potency, enteric-coated pancreatic enzymes remains the main therapy for the treatment of steatorrhea in the majority of patients with idiopathic and alcoholic pancreatitis.
    2. Vitamin D and calcium malabsorption leads to osteopenia and tetany. Vitamin K is also malabsorbed, but bleeding is rare. This malabsorption is thought to be due to the failure of R factor to cleave from the vitamin B12-intrinsic factor complex, resulting in failure to absorb vitamin B12.
Hereditary Pancreatitis and Shwachman's Syndrome
  1. Hereditary Pancreatitis an autosomal dominant bimodal incidence with peaks at about 10 and 17 years of age.
  2. Pancreatic calcification develops early, in the first decade, but in most respects, clinical course is similar to that of chronic pancreatitis of other causes.
  3. Children are from an affected family or have a structural abnormality in the pancreas or biliary tree (particularly pancreas divisum or choledochal cyst).
  4. The association of pancreatic malabsorption, steatorrhoea, and failure to thrive, with metaphyseal  dysplasia, neutropenia and other haematological abnormalities, is known as Shwachman's syndrome and accounts for a high proportion of children presenting with painless pancreatic insufficiency.
  5. It is probably inherited as an autosomal recessive condition. With appropriate dietary modification and enzyme supplements, the prognosis is good. 
Pancreatic Pseudocyst
Pancreatic pseudocysts are a relatively common complication of pancreatitis, especially alcoholic pancreatitis. Pseudocysts are localized collections of fluid and contain concentrations of pancreatic enzymes; they are usually confined to the retroperitoneal areas by a fibrous membrane that is devoid of endothelial lining. They develop after 15% to 50% of all attacks of acute pancreatitis, many resolving spontaneously, and are seen in 20% to 40% of patients with chronic pancreatitis.

The D' EGIDIO classification of Pancreatic Pseudocyst
Context Pancreatic Duct Duct-pseudocyst
Type I Acute post-necrotic
Normal No Percutaneous drainage
Type II Acute-on Chronic
Abnormal (no stricture) 50:50 Internal drainage or resection
Type III Chronic pancreatitis Abnormal (stricture) Yes Internal drainage with duct decompression
  • Investigation of choice – CT – Scan
  1. They typically result from disruption of the pancreatic duct with extravasation of pancreatic enzymes.
  2. Although outside the peritoneal cavity, pseudocysts may migrate to the mediastinum or pelvis, and associated pleural effusions also occur. Ultrasound has demonstrated that such cysts represent a very common complication although rarely it warrants intervention.
  3. Most lesions of 5cm or less resolve spontaneously. When larger, pseudocysts may cause pain, gastrointestinal obstruction or a palpable mass.
  4. Pseudocyst fluid – similar electrolyte concentrations to plasma, Higher concentration of amylase, lipose and enterokinase.
  5. Most common symptom – pain  & Most common complication – infection
  • Other are
  1. GI obstruction                  
  2. Perforation                      
  3. Hemorrhage
  4. Thrombosis (Splenic vein) Most common                  
  5. Pseudoaneurysm (Splenic artery) Most common
Pseudocyst Complications
· Infection (MC} · Duodenal obstruction
· Pain due to expansion · Rupture
· Hemorrhage · Abscess

Indications for drainage
  1. Symptomatic > 6 weeks                                            
  2. Size > 6 cm
  3. Complications                                                            
  4. Suspicion of malignancy
Benign Malignant Functional Non Functional (15-25%) Primary
Cystadenoma (middle aged/older women, <10% of pancreatic lesion)
Solid/papillary neoplasm (F>>m-,10-35yrs, rare, locally invasive) 
Ductal adenocarcinoma (90%) (M.C.)
Cystadeno carcinoma (F>M,40 to 60 yrs,<2%)
Acinar cell carcinoma
Rare, F=M, PAS+ve)
Insulinoma (MC, Endocrine tumor)
Gastrinoma (Zollinger-Ellision Syndrome)
VI Poma (Verner Morrison Syndrome)
Secrete Pancreatic polypeptide higher malignancy rate (90%) Primary involvement with non Hodgkin’s lymphoma, rare (M=F)

Carcinoma Of The Pancreas
  1. Most common site is head of the pancreas
  2. Most common histopathology is intraductal type of adeno carcinoma.
  3. Of pancreatic tumors, 95% develop from the exocrine portion of the pancreas, including the ductal epithelium, acinar cells, connective tissue, and lymphatic tissue.
  4. Approximately 15-20% occur in the body of the pancreas, and 5-10% occur in the tail.
  5. Pancreatic cancer typically first metastasizes to regional lymph nodes, then to the liver and, less commonly, to the lungs.
  6. The male-to-female ratio 1.2-1.5:1.
  7. Clinical feature: Patients typically report the gradual onset of anorexia, malaise, nausea, and midepigastric abdominal pain. Significant weight loss is a characteristic feature of pancreatic cancer.
  8. Pain is the most common presenting symptom in patients with pancreatic cancer. Back radiation of the pain indicating retroperitoneal invasion of the splanchnic nerve plexus by the tumor.
  9. The most characteristic sign of head pancreatic carcinoma, is obstructive jaundice.
  10. Patients with jaundice may have a palpable gallbladder (ie, Courvoisier sign) and may have evidence of excoriations from pruritus.
Risk factor –
  • Age, - 90% - >55yrs
  • Male gender,
  • Smoking,
  • Diet low in vegetable and fruits,
  • Obesity,
  • DM,
  • Chronic pancreatitis, 
  • H. pyloric infection.
Extra Edge: Alcohol is not a cause of CA pancreas.
  1. Pathology
  1. 70% of cases are in Head, 20% in the body and 10% in tail
  2. Microscopic appearance can be mucinous or non-mucous secreting
  3. Mutations in K-ras gene have been found in >85% cases
  4. Mutation of the pl64 gene located on 9p21 (a gene also implicated in malignant melanoma)
  5. Mutation in P53 gene is associated in 75% of cases
  6. Hereditary pancreatitis significantly increases the risk
  1. Lab Studies:
    1. General laboratory studies
    2. Tumor markers
      1. Carcinoembryonic antigen (CEA) is a high molecular weight glycoprotein found normally in fetal tissues. The reference range is < 2.5 mg/mL. Only 40-45% of patients with pancreatic carcinoma have elevations in CEA levels.
      2. CA 19-9 is a murine monoclonal antibody originally made against colorectal cancer cells. The reference ange of CA 19-9 is < 37 U/mL. In pancreatic carcinoma, 75-85% have elevated CA 19-9 levels.
      3. In the absence of biliary obstruction or benign pancreatic disease, a CA 19-9 value greater than 100 U/mL is highly specific for malignancy, usually pancreatic.
  2. Imaging Studies:
    Most sensitive investigative EUS
    Endoscopic ultrasound: EUS has detection rates of 99-100% for pancreatic carcinomas.
    CT-Scan is the investigation of choice.
    1. Computed tomography scan: Standard abdominal CT scan can detect 70-80% of pancreatic carcinomas. CT scans can be used to direct fine-needle aspiration of masses.
    2. Percutaneous ultrasound: Percutaneous abdominal ultrasonography is useful for patients with pancreatic cancer who present with jaundice, by detecting intrahepatic bile duct dilation/extrahepatic biliary obstruction.
    3. Cholangiopancreatography (MRCP) should usually be performed to definitively diagnose the source of extrahepatic obstruction.
    4. Endoscopic retrograde cholangiopancreatography: Brush cytology and forceps biopsy at the time of ERCP have been used to histologically diagnose pancreatic carcinoma.
    5. Magnetic resonance imaging
    6. Histologic Findings: 80% are adenocarcinomas of the ductal epithelium. Only 2% of tumors of the exocrine pancreas are benign.
  3. Treatment:
  • The only therapy that has definitively been shown to increase the survival of patients with pancreatic cancer is surgical resection. The mean survival for patients with unresectable disease remains 4-6 months.
  1. Surgical Care: Only 20% of patients present with resectable disease.
    Pancreaticoduodenectomy (Whipple operation)
    1. The standard operation for carcinoma of the head of the pancreas is a pancreaticoduodenectomy (ie, Whipple procedure).
    2. This operation involves en bloc resection of the pancreatic head; the first, second, and third portions of the duodenum; the distal antrum; and the distal common bile duct.
    3. The patient's gastrointestinal tract is reconstructed with a gastrojejunostomy.
    4. The common bile duct and residual pancreas are anastomosed into a segment of small bowel.
    5. A more recent variation of the operation spares the pylorus, allowing for a more natural physiologic emptying of the stomach.
    6. Some surgeons prefer total pancreatectomy to avoid the risks of anastomotic leaks and pancreatic fistulas. This has the disadvantage of leaving the patient with brittle diabetes postoperatively.
  2. Chemotherapy
    1. Pancreatic carcinoma has been markedly resistant to chemotherapeutic regimens, either alone or in combination therapy.
    2. The most active agents have been 5-fluorouracil (5-FU) and the more recently gemcitabine and  Erlotinib

  1. Chemoradiation 5 Floururacil + External beam radiotherapy
    1. 5 FU acts as a radiosensitizing agent
    2. Can also be used as ‘neoadjuvant’ chemoradiation before surgery
    3. Has increased the survival rates
  2. Intraoperative Radiation therapy
    1. Has the potential to deliver high does of radiation to the tumor while sparing neighbouring tissue does not give better results than external beam treatment
  3. Gemcitabine
    1. Deoxycytidine analogue
    2. Duration of survival only moderately improved
    3. Improvement in quality of life significant
  4. Prognosis
    Curative operation possible in only 10-15%
    5 year survival rate following such operations is 10%
    Median survival for patients having unresectable tumor is 6 months.
Endocrine Pancreatic Tumours
  1. Zollinger-Ellison syndrome (also termed gastrinoma syndrome)
  2. Verner-Morrison syndrome,
  3. WDHA (watery diarrhea, hypokalemia, and achlorhydria) syndrome,
  4. Pancreatic cholera (also termed [VIP]–releasing tumor or VIPoma);
  5. Glucagonoma syndrome.
  6.  Somatostatinoma syndrome.
    1. The cells in pancreatic endocrine neoplasms are termed amine precursor uptake and decarboxylation  (APUD) cells because they have a high amine content, are capable of amine precursor uptake, and contain an amino acid decarboxylase.
    2. The tumors arise from APUD stem cells, which are pluripotential neuroendocrine cells located within the ductular epithelium of the exocrine pancreas and elsewhere in the distal foregut
  1. Insulinoma:
    1. Most common among of neoplasm of endocrine pancreas is insulinoma.
    2. Insulinoma: Insulinomas are insulin-secreting tumors associated with the Whipple triad. The triad includes
      1. Symptoms of fasting hypoglycemia,
      2. Documented fasting hypoglycemia with a serum glucose < 50 mg/dL
      3. Relief of hypoglycemic symptoms after glucose administration.
  1. Gastrinoma: The classic triad of Zollinger-Ellison syndrome includes
    1. Severe gastrointestinal ulcerative disease,
    2. Gastric acid hypersecretion
    3. Nonbeta islet cell tumors of the pancreas (Zollinger, 1955). Abdominal pain and peptic ulceration of the upper gastrointestinal tract are the most common symptoms and are observed in 90-95% of patients with Zollinger-Ellison syndrome.
      (Fasting serum gastrin test: Levels greater than 200 pg/mL are suggestive of gastrinoma, and levels greater than 1000 pg/mL are virtually diagnostic of gastrinoma)
  2. VIPoma: Symptoms of Verner-Morrison or WDHA syndrome (ie, watery diarrhea, hypokalemia, achlorhydria, acidosis) are the result of the physiologic effects of overproduction of VIP by pancreatic endocrine neoplasms.
    1. The primary symptom of patients with a VIPoma is watery diarrhea. Abdominal
    2. Abdominal cramps are common, and flushing episodes may occur. (The level of serum VIP ranges from 225-1850 pg/mL. The normal serum VIP level is <170 pg/mL)
  1. Glucagonoma: Glucagonomas secrete excessive amounts of glucagon and cause a syndrome characterized by dermatitis, stomatitis, weight loss, anemia. The dermatitis associated with glucagonoma syndrome is termed necrolytic migratory erythema.
    This dermatitis is characterized by the cyclic migration of erythematous patches that spread serpiginously and then reveal central points of healing. Patients with glucagonoma syndrome have secondary thromboembolic phenomena; therefore, they may have a history consistent with deep venous thrombosis and/or pulmonary embolism. (Serum glucagon levels >1000 pg/mL are diagnostic, levels less than 150 pg/mL are normal)
  1. Somatostatinoma: Patients often have diabetes mellitus, which is probably secondary to the inhibitory action of somatostatin on insulin and glucagon release. Inhibition of the action of cholecystokinin by somatostatin causes relative biliary stasis and the formation of gallbladder calculi. Patients may also have diarrhea and/or steatorrhea.
  2. MEN 1 syndrome, or Wermer syndrome, is an autosomal dominant disorder. The syndrome is characterized by hyperparathyroidism, adenomas of pituitary, and neoplasms of endocrine pancreas.
    Imaging Studies:
    1. Localization study of choice for insulinoma – intraoperative USG
    2. Localization study of choice for gastrinoma – somatostatin receptor scintigraphy.
    3. High-resolution contrast-enhanced spiral CT scanning with thin sections is the initial imaging technique used to localize most neoplasms of the endocrine pancreas.
    4. Magnetic resonance imaging
    5. Somatostatin receptor scintigraphy: Radiolabeled octreotide is a somatostatin analogue that preferentially binds to somatostatin receptors; the intravenous administration of octreotide can be used to identify such tumors.
  • Medical Care: 
  1. Upon initial presentation, patients with insulinoma may require immediate potassium replacement and dextrose administration.
  2. The primary initial concern in the treatment of a patient who presents first with VIPoma-associated diarrhea is the replacement of volume losses and the correction of acid-base and electrolyte abnormalities.
  3. Patients with glucagonomas requires preoperative control of hyperglycemia.
  • Surgical Care:
  1. Surgical management of the primary tumor is similar for the different types of pancreatic endocrine neoplasms:
  2. Small benign lesions remote from the main pancreatic duct can be enucleated.
  3. Tumors deep in the substance of the pancreatic gland, and therefore close to the main duct, have ill-defined capsules, and tumors larger than 2 cm in diameter should be treated with regional pancreatectomy.
  4. Tumors in the tail of the pancreas can be managed with distal pancreatectomy
  5. Lesions in the head or uncinate process of the pancreas can be resected with pancreaticoduodenectomy.
  1. Periampullary Carcinoma
    1. Obstructive jaundice is the usual presenting feature, and ultrasound (especially endoscopic) or a CT scan may demonstrate the lesion.
    2. At ERCP, the endoscopic appearance is immediately suggestive of a malignant tumour at the ampulla, and the absence of extension along the pancreatic duct or bile duct helps in the distinction from an infiltrating pancreatic carcinoma or extension of a cholangiocarcinoma.
    3. Biopsies, taken at endoscopy or ERCP, should confirm the diagnosis.
    4. Ampullary neoplasms are usually papillary or solid tumours which invade locally. The usual histological pattern is of moderately well-differentiated adenocarcinoma.
  2. Ampullary Carcinoma
    1. The periampullary region is anatomically complex, representing the junction of 3 different epithelia, pancreatic ducts, bile ducts, and duodenal mucosa.
    2. Carcinomas originating in the ampulla of Vater by gross inspection can arise from 1 of 4 epithelial types
      1. Terminal common bile duct,
      2. Duodenal mucosa,
      3. Pancreatic duct,
      4. Ampulla of Vater.
        In general, ampullary cancers produce sialomucins, whereas periampullary tumors secrete sulfated mucins.
  • Clinical Feature:
  1. Jaundice it the most common presenting symptoms. Jaundice may be intermittent Waxing & waning type.
  2. Patients with carcinoma of the ampulla of Vater often complain of anorexia, nausea, vomiting, jaundice,  pruritus, or weight loss, abdominal pain.
  3.  Diarrhea may be associated with an absence of lipase in gut because of pancreatic duct obstruction.
  4. Physical:  
    1. Some patients might demonstrate a distended, palpable Courvoisier gallbladder (ie, palpable gall bladder in a patient with jaundice).
    2. A rising bilirubin level due to obstructive jaundice often is the sole presenting symptom.
  • Investigation:
  1. Ultrasound of the abdomen is the initial study (dilation of these ducts essentially is diagnostic for extrahepatic obstruction).
  2. CT scan often demonstrates a mass but is not helpful in differentiating ampullary carcinoma from tumors of the head of the pancreas or periampullary region.
  3.  Both CT scan and ultrasound findings can help reveal metastatic disease in the liver or regional lymph nodes
  4. ERCP can show irregular pancreatic duct narrowing, displacement of the main pancreatic duct, destruction or displacement of the side branches of the duct, and pooling of contrast material in necrotic areas of tumor.
  5. Lab Studies: Routine laboratory studies include a complete blood cell count, electrolyte panel, liver function studies (prothrombin time, bilirubin [direct and indirect], transaminases, alkaline phosphatase), CEA, and CA 19-9 (CA 19-9 and CEA is often is elevated in pancreatic malignancies and might have a role in assessing response to therapy or predicting tumor recurrence). 
Extra Edge:
  • Most sensitive investigation endoscopic USG
  • Most sensitive Non invasive investigation MRCP.
  • Investigation of choice CT-Scan.
Stage I -  Vegetating tumor limited to the epithelium with no involvement of the sphincter of Oddi
Stage II - Tumor localized in the duodenal submucosa without involvement of the duodenal muscularis propria but possible involvement of the sphincter of Oddi
Stage III -  Tumor of the duodenal muscularis propria
Stage IV -  Tumor of the periduodenal area or pancreas, with proximal or distal lymph node Involvement
  1. Surgical Care: The standard surgical approach is pancreaticoduodenal resection (Whippl procedure).
  2. The procedure involves en bloc resection of the gastric antrum and duodenum; a segment of the first portion of the jejunum, gallbladder, and distal common bile duct; the head and often the neck of the pancreas; and adjacent regional lymph nodes. .
  3. For patients with unresectable disease, endoscopic stenting to achieve biliary decompression is an appropriate palliative procedure. 

Test Your Skills Now!
Take a Quiz now
Reviewer Name