Diseases of the Pancreas

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Diseases of the Pancreas

• Victor Politi, M.D., Medical Director
• SVCMC, School of Allied Health Professions,
  Physician Assistant Program

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Pancreas anatomy

• The pancreas is an elongated, tapered organ
  located across the back of the abdomen, behind
  the stomach.
• The right side of the organ (called the head) is
  the widest part of the organ and lies in the
  curve of the duodenum (the first section of the
  small intestine).
• The tapered left side extends slightly upward
  (called the body of the pancreas) and ends near
  the spleen (called the tail).

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Pancreas anatomy

• The pancreas is made up of two types of tissue
• exocrine tissue
• The exocrine tissue secretes digestive enzymes.
  These are secreted into a network of ducts that
  join the main pancreatic duct, which runs the
  length of the pancreas.
• endocrine tissue
• The endocrine tissue, which consists of the
  islets of Langerhans, secretes hormones into the
  bloodstream.

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• Accessory duct of Santorini
• Duct of Wirsung

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Pancreas anatomy

• The pancreas has digestive and hormonal
  functions
• The enzymes secreted by the exocrine tissue in
  the pancreas help break down carbohydrates, fats,
  and proteins in the duodenum.
• These enzymes travel down the pancreatic duct
  into the bile duct in an inactive form.
• When they enter the duodenum, they are activated.
  
• The exocrine tissue also secretes bicarbonate to
  neutralize stomach acid in the duodenum.

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Pancreas anatomy

• The hormones secreted by the endocrine tissue in
  the pancreas are insulin, glucagon (which
  regulate the level of glucose in the blood),
  somatostatin (which prevents the release of the
  other two hormones), and many others.

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What is Pancreatitis?

• Pancreatitis is an inflammatory process in which
  pancreatic enzymes autodigest the gland

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• Normally, digestive enzymes do not become active
  until they reach the small intestine, where they
  begin digesting food.
• But if these enzymes become active inside the
  pancreas, they start "digesting" the pancreas
  itself

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• The gland can sometimes heal without any
  impairment of function or any morphologic
  changes.
• This process is known as acute pancreatitis.
• It can recur intermittently, contributing to the
  functional and morphologic loss of the gland.
• Recurrent attacks are referred to as chronic
  pancreatitis.

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• Acute pancreatitis occurs suddenly and lasts for
  a short period of time and usually resolves.
• Chronic pancreatitis does not resolve itself and
  results in a slow destruction of the pancreas.

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• Either form can cause serious complications.
• In severe cases, bleeding, tissue damage, and
  infection may occur.
• Pseudocysts, accumulations of fluid and tissue
  debris, may also develop.
• Enzymes and toxins may enter the bloodstream,
  injuring the heart, lungs, and kidneys, or other
  organs.

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Acute edematous pancreatitis

• Since the pancreas is located in the
  retroperitoneal space with no capsule
  -inflammation can spread easily.
• In acute pancreatitis, parenchymal edema and
  peripancreatic fat necrosis occur first.
• This process is known as acute edematous
  pancreatitis

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Necrotizing pancreatitis

• When necrosis involves the parenchyma,
  accompanied by hemorrhage and dysfunction of the
  gland, the inflammation evolves into hemorrhagic
  or necrotizing pancreatitis

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Necrotizing pancreatitis

• Pseudocysts and pancreatic abscesses can result
  from necrotizing pancreatitis because of enzymes
  being walled off by granulation tissue (ie,
  pseudocyst formation) or bacterial seeding of
  pancreatic or peripancreatic tissue (ie,
  pancreatic abscess formation).
• An ultrasound or, preferably, a CT scan can be
  used detect both.

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• The inflammatory process can cause systemic
  effects because of the presence of cytokines,
  such as bradykinins and phospholipase A.
• These cytokines may cause vasodilation, increase
  in vascular permeability, pain, and leukocyte
  accumulation in the vessel walls.
• Fat necrosis may cause hypocalcemia.
• Pancreatic B cell injury may lead to
  hyperglycemia.

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Mortality/Morbidity

• Although acute pancreatitis should be noted,
  chronic pancreatitis has a more severe
  presentation as episodes recur.
• Acute respiratory distress syndrome (ARDS), acute
  renal failure, cardiac depression, hemorrhage,
  and hypotensive shock all may be systemic
  manifestations of acute pancreatitis in its most
  severe form.

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Acute Pancreatitis

• Some people have more than one attack and recover
  completely after each, but acute pancreatitis can
  be a severe, life-threatening illness with many
  complications.

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Acute Pancreatitis

• About 80,000 cases occur in the United States
  each year some 20 percent of them are severe.
• Acute pancreatitis occurs more often in men than
  women.

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• The risk for African American persons aged 35-64
  years is 10 times higher than for any other
  group.
• African American persons are at higher risk than
  white persons in that same age group

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History

• The main presentation of acute pancreatitis is
  epigastric pain or right upper quadrant pain
  radiating to the back
• The pain may be severe and may become
  constant--just in the abdomen-or it may reach to
  the back and other areas.
• It may be sudden and intense or begin as a mild
  pain that gets worse when food is eaten.

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History

• Nausea and/or vomiting
• Fever
• Query the patient about recent surgeries and
  invasive procedures (ie, endoscopic retrograde
  cholangiopancreatography) or family history of
  hypertriglyceridemia.
• Patients frequently have a history of previous
  biliary colic and binge alcohol consumption, the
  major causes of acute pancreatitis.

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Physical

• Tachycardia
• Tachypnea
• Hypotension
• Fever
• Abdominal tenderness, distension, guarding, and
  rigidity

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Physical

• Mild jaundice
• Diminished or absent bowel sounds
• Because of contiguous spread of inflammation
  (effusion) from the pancreas, lung auscultation
  may reveal basilar rales, especially in the left
  lung.
• Occasionally, in the extremities, muscular spasm
  may be noted secondary to hypocalcemia.

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Physical

• Severe cases may have a Grey Turner sign (ie,
  bluish discoloration of the flanks) and Cullen
  sign (ie, bluish discoloration of the
  periumbilical area) caused by the retroperitoneal
  leak of blood from the pancreas in hemorrhagic
  pancreatitis.

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• This is Grey-Turner's sign with haemorrhage
  appearing in both flanks. It is due to extensive
  retro-peritoneal bleeding and typically occurs in
  haemorrhagic pancreatitis

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Causes

• The major causes are long-standing alcohol
  consumption and biliary stone disease.

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Causes

• In developed countries, the most common cause of
  acute pancreatitis is alcohol abuse
• On the cellular level, ethanol leads to
  intracellular accumulation of digestive enzymes
  and their premature activation and release.
• On the ductal level, ethanol increases the
  permeability of ductules, which allow enzymes to
  reach the parenchyma, resulting in pancreatic
  damage

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Causes

• Ethanol increases the protein content of the
  pancreatic juice and decreases bicarbonate levels
  and trypsin inhibitor concentrations. This leads
  to the formation of protein plugs that block the
  pancreatic outflow and obstruction

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Causes

• Another major cause of acute pancreatitis is
  biliary stone disease (eg, cholelithiasis,
  choledocholithiasis).
• A biliary stone may lodge in the pancreatic duct
  or ampulla of Vater and obstruct the pancreatic
  duct, leading to extravasation of enzymes into
  the parenchyma.

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Minor causes of acute pancreatitis

• Medications,
• including azathioprine, corticosteroids,
  sulfonamides, thiazides, furosemides, NSAIDs,
  mercaptopurine, methyldopa, and tetracyclines
• Endoscopic retrograde cholangiopancreatography
  (ERCP)
• Hypertriglyceridemia
• (When the triglyceride (TG) level exceeds 1000
  mg/U, an episode of pancreatitis is more likely.)
• Peptic ulcer disease

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Minor causes of acute pancreatitis

• Abdominal or cardiopulmonary bypass surgery
• may insult the gland by ischemia
• Trauma to the abdomen or back
• resulting in sudden compression of the gland
  against the spine posteriorly
• Carcinoma of the pancreas
• which may lead to pancreatic outflow obstruction
• Viral infections, including mumps,
  Coxsackievirus, cytomegalovirus (CMV), hepatitis
  virus, Epstein-Barr virus (EBV), and rubella
• Bacterial infections
• such as mycoplasma

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Minor causes of acute pancreatitis

• Intestinal parasites, such as ascaris, which can
  block the pancreatic outflow
• Pancreas divisum
• Scorpion and snake bites
• Vascular factors, such as ischemia or vasculitis

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Other problems to be considered

• Perforated viscus
• Acute peritonitis
• Choledocholithiasis
• Macroamylasemia
• Macrolipasemia
• Intestinal obstruction
• Pancreatic cancer
• Malabsorption syndromes/processes

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Acute Pancreatitis - Diagnosis

• History
• Physical exam
• Lab Studies
• During acute attacks, the blood contains at least
  three times more amylase and lipase than usual.
  Amylase and lipase are digestive enzymes formed
  in the pancreas.
• Changes may also occur in blood levels of
  glucose, calcium, magnesium, sodium, potassium,
  and bicarbonate.
• After the pancreas improves, these levels usually
  return to normal.

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Acute Pancreatitis - Diagnosis

• Imaging Studies
• X-ray
• ultrasound
• CT

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Lab Studies

• A complete blood count (CBC) demonstrates
  leukocytosis (WBC gt12000) with the differential
  being shifted towards the segmented polymorphs.
• If blood transfusion is necessary, as in cases of
  hemorrhagic pancreatitis, obtain type and
  crossmatch.
• Measure blood glucose level because it may be
  elevated from B cell injury in the pancreas.
• Obtain measurements for BUN, creatine (Cr), and
  electrolytes (Na, K, Cl, CO2, P, Mg) a great
  disturbance in the electrolyte balance is usually
  found, secondary to third spacing of fluids

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Lab Studies

• Measure amylase levels, preferably the Amylase P,
  which is more specific to pancreatic pathology.
  Levels more than 3 times higher than normal
  strongly suggest the diagnosis of acute
  pancreatitis
• Lipase levels also are elevated and remain high
  for 12 days. In patients with chronic
  pancreatitis (usually caused by alcohol abuse),
  lipase may be elevated in the presence of a
  normal serum amylase level

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Lab Studies

• Perform liver function tests (eg, alkaline
  phosphatase, serum glutamic-pyruvic transaminase
  SGPT, serum glutamic-oxaloacetic transaminase
  SGOT, G-GT) and bilirubin, particularly with
  biliary origin pancreatitis.
• In chronic pancreatitis the enzymes may be normal
  or low due to pancreas burn out

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Imaging Studies

• Perform a plain KUB (Kidneys, ureters, bladder)
  with the patient in the upright position to
  exclude viscus perforation (ie, air under the
  diaphragm).
• In cases with a recurrent episode of chronic
  pancreatitis, peripancreatic calcifications may
  be noted.

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Imaging Studies

• Ultrasound
• can be used as a screening test.
• If overlying gas shadows secondary to bowel
  distention are present, it may not be specific.

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Imaging Studies

• CT scan is the most reliable imaging modality in
  the diagnosis of acute pancreatitis.

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Pancreatitis, Acute - CT Scan
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Pancreatitis, Chronic - CT Scan
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Treatment

• Treatment depends on the severity of the attack.
• If no kidney or lung complications occur, acute
  pancreatitis usually improves on its own.
• Treatment, in general, is designed to support
  vital bodily functions and prevent complications.

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Treatment

• Most of the cases presenting to the ED are
  treated conservatively, and approximately 80
  respond to such treatment

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Treatment

• Fluid resuscitation
• Monitor accurate intake/output and electrolyte
  balance of the patient.
• Crystalloids are used, but other infusions, such
  as packed red blood cells (PRBCs), are
  occasionally administered, particularly in the
  case of hemorrhagic pancreatitis.
• Central lines and Swan-Ganz catheters are used in
  patients with severe fluid loss and very low
  blood pressure.

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Treatment

• Patients should have nothing by mouth, and a
  nasogastric tube should be inserted to assure an
  empty stomach and to keep the GI system at rest.
• Begin parenteral nutrition if the prognosis is
  poor and if the patient is going to be kept in
  the hospital for more than 4 days.

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Treatment

• Analgesics are used to relieve pain. Meperidine
  is preferred over morphine because of the greater
  spastic effect of the latter on the sphincter of
  Oddi.
• Antibiotics are used in severe cases associated
  with septic shock or when the CT scan indicates
  that a phlegmon of the pancreas has evolved.

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Treatment

• Other conditions, such as biliary pancreatitis
  associated with cholangitis, also need antibiotic
  coverage.
• The preferred antibiotics are the ones secreted
  by the biliary system, such as ampicillin and
  third generation cephalosporins.

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Treatment

• Continuous oxygen saturation should be monitored
  by pulse oxymetry and acidosis should be
  corrected. When tachypnea and pending respiratory
  failure develops, intubation should be performed.
  
• Perform CT-guided aspiration of necrotic areas,
  if necessary.
• An ERCP may be indicated for common duct stone
  removal

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Treatment

• Surgical Consult
• For phlegmon of the pancreas
• Hemorrhagic pancreatitis
• Patients who fail to improve despite optimal
  medical treatment
• Patients who push the Ranson score even further
• Biliary pancreatitis

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Medications

• Antibiotics
• Used to cover the microorganisms that may grow in
  biliary pancreatitis and acute necrotizing
  pancreatitis.
• The empiric antibiotic regimen usually is based
  on the premise that enteric anaerobic and aerobic
  gram-bacilli microorganisms are often the cause
  of pancreatic infections.
• Once culture sensitivities are made, adjustments
  in the antibiotic regimen can be done.

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• Antibiotics
• Ceftriaxone (Rocephin), Unasyn, Mefoxitin
• Ampicillin (Marcillin, Omnipen),Gent, Flagyl
• Analgesics
• Meperidine (Demerol)

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Ranson Scale

• Ranson developed a series of different criteria
  for the severity of acute pancreatitis
• For the following catagories-
• answer each question regarding the patient then
  add up total score for prognosis
• If answer is no (o point)
• If answer is yes (1 point)

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Ranson Scale

• Present on admission
• Older than 55 years
• WBC higher than 16,000 per mcL
• Blood glucose higher than 200 mg/dL
• Serum lactate dehydrogenase (LDH) more than 350
  IU/L
• SGOT (ie, aspartate aminotransferase AST)
  greater than 250 IU/L

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Ranson Scale

• Developing during the first 48 hours
• Hematocrit fall more than 10
• BUN increase more than 8 mg/dL
• Serum calcium less than 8 mg/dL
• Arterial oxygen saturation less than 60 mm Hg
• Base deficit higher than 4 mEq/L
• Estimated fluid sequestration higher than 600 mL

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Ranson Score

• A Ranson score of 0-2 has a minimal mortality
  rate.
• A Ranson score of 3-5 has a 10-20 mortality
  rate.
• A Ranson score higher than 5 has a mortality rate
  of more than 50 and is associated with more
  systemic complications

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Complications

• Infected pancreatic necrosis may result from
  seeding of bacteria into the inflammation.
• An acute pseudocyst is an effusion of pancreatic
  juice that is walled off by granulation tissue
  after an episode of acute pancreatitis.
• Hemorrhage into the GI tract retroperitoneum or
  the peritoneal cavity is possible because of
  erosion of large vessels.
• Intestinal obstruction or necrosis may occur.

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Other Disorders of the Pancreas
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Pancreatic Cancer

• Pancreatic cancer is the fourth most common
  cancer in men and women in the US, according to
  the American Cancer Society.
• The majority of pancreatic cancer occurs in
  people 50 years of age or older

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• In the United States, approximately 30,000 people
  die of pancreatic cancer each year.
• Among cancers of the gastrointestinal tract, it
  is the third most common malignancy and the fifth
  leading cause of cancer-related mortality.

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• About 95 of cancerous tumors of the pancreas are
  adenocarcinomas.
• Adenocarcinomas usually originate in the
  glandular cells lining the pancreatic duct.
• Most adenocarcinomas occur in the head of the
  pancreas, the part nearest the first segment of
  the small intestine (duodenum).

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• Adenocarcinoma usually does not develop before
  age 50 the average age at diagnosis is 55.
• These tumors are nearly twice as common in men as
  in women and are slightly more common in blacks
  than in whites.
• Adenocarcinoma of the pancreas is 2 to 3 times
  more common in heavy smokers than in nonsmokers.
• People with chronic pancreatitis are at greater
  risk as well

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• The disease is difficult to diagnose in its early
  stages, and most patients have incurable disease
  by the time they present with symptoms.
• The overall 5-year survival rate for this disease
  is less than 5.

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• Pancreatic cancers can arise from both the
  exocrine and endocrine portions of the pancreas.
• Of pancreatic tumors, 95 develop from the
  exocrine portion of the pancreas, including the
  ductal epithelium, acinar cells, connective
  tissue, and lymphatic tissue.
• Approximately 75 of all pancreatic carcinomas
  occur within the head or neck of the pancreas

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• Typically, pancreatic cancer first metastasizes
  to regional lymph nodes, then to the liver, and
  less commonly, to the lungs. It can also directly
  invade surrounding visceral organs such as the
  duodenum, stomach, and colon.

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• As in other organs, chronic inflammation is a
  predisposing factor in the development of
  pancreatic cancer.
• Patients with chronic pancreatitis from alcohol,
  especially those with familial forms, have much
  higher incidence and an earlier age of onset of
  pancreatic carcinoma.

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Cystadenocarcinoma

• Cystadenocarcinoma of the pancreas is a rare type
  of pancreatic cancer that develops from a
  fluid-filled noncancerous tumor called a
  cystadenoma.
• It often causes upper abdominal pain and may grow
  large enough for a doctor to feel it through the
  abdominal wall.

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Mortality/Morbidity

• Pancreatic carcinoma is unfortunately usually a
  fatal disease.
• Most patients eventually succumb to the
  consequences of local invasion and metastatic
  cancer, and true long-term cures are rare.
• Endocrine and cystic neoplasms of the pancreas
  have much better survival rates than pancreatic
  adenocarcinoma.

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History

• Unfortunately, the initial symptoms are often
  quite nonspecific and subtle in onset.
• Patients typically report the gradual onset of
  nonspecific symptoms such as anorexia, malaise,
  nausea, fatigue, and midepigastric or back pain.
• Significant weight loss is a characteristic
  feature of pancreatic cancer.

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History

• Pain is the most common presenting symptom in
  patients with pancreatic cancer.
• Typically, it is midepigastric in location, with
  radiation of the pain sometimes occurring to the
  mid- or lower-back region.

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History

• The most characteristic sign of pancreatic
  carcinoma of the head of the pancreas is painless
  obstructive jaundice.
• Patients with this sign may come to medical
  attention before their tumor grows large enough
  to cause abdominal pain.
• Pruritus may accompany obstructive jaundice.

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History

• Migratory thrombophlebitis (ie, Trousseau sign)
  and venous thrombosis also occur with higher
  frequency in patients with pancreatic cancer.
• Depression is reported to be more common in
  patients with pancreatic cancer than in patients
  with other abdominal tumors.
• In some patients, depression may be the most
  prominent presenting symptom.

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Physical

• The physical examination findings in a patient
  with pancreatic cancer are usually limited to
  evidence of significant weight loss and some
  mild-to-moderate midepigastric tenderness.
• Patients with jaundice may have a palpable
  gallbladder (ie, Courvoisier sign) and may have
  evidence of skin excoriations from pruritus.

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Physical

• Patients presenting with end-stage disease may
  have ascites, a palpable abdominal mass,
  hepatomegaly from liver metastases, or
  splenomegaly from portal vein obstruction.

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Labs

• The laboratory findings in patients with
  pancreatic cancer are usually nonspecific.
• As with many chronic diseases, a mild
  normochromic anemia may be present.
• Thrombocytosis is also sometimes observed in
  patients with cancer.
• The major useful tumor marker for pancreatic
  carcinoma is carbohydrate antigen 19-9 (CA 19-9).
  

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• Gross section of an adenocarcinoma of the
  pancreas measuring 5 X 6 cm resected from the
  pancreatic body and tail. Although the tumor was
  considered to have been fully resected and had
  not spread to any nodes, the patient died of
  recurrent cancer within 1 year

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• CTshowing a pancreatic adenocarcinoma of the
  pancreatic head. The gallbladder (gb) is
  distended because of biliary obstruction. The
  superior mesenteric artery (sma) is surrounded by
  tumor, making this an unresectable T4 lesion.

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Treatment

• The only therapy that has definitively been shown
  to increase the survival of patients with
  pancreatic cancer is surgical resection.
• For patients with disease not amenable to
  curative resection, little has been shown to
  significantly impact survival.
• The mean survival for patients with unresectable
  disease remains 4-6 months.

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Treatment

• Other therapies for pancreatic cancer should
  include palliation of the major symptoms of
  disease.
• Chemotherapy
• Radiation therapy

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Pancreaticoduodenectomy (Whipple operation)

• The standard operation for carcinoma of the head
  of the pancreas is a pancreaticoduodenectomy
  (Whipple procedure).
• This operation involves resection of the
  pancreatic head the first, second, and third
  portions of the duodenum the distal antrum and
  the distal common bile duct

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Deterrence/Prevention

• Smoking is the most significant reversible risk
  factor for pancreatic cancer. Estimates indicate
  that smoking accounts for up to 30 of cases of
  pancreatic cancer.
• A diet high in energy intake and low in fresh
  fruits and vegetables increases the risk of
  pancreatic cancer.
• Alcohol consumption does not increase the risk of
  pancreatic cancer unless it leads to chronic
  pancreatitis. A multicenter study of more than
  2000 patients with chronic pancreatitis showed a
  26-fold increase in the risk of developing
  pancreatic cancer.

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Prognosis

• The mean survival for patients with unresectable
  disease remains 4-6 months, with a 5-year
  survival rate of less than 3.
• The median survival for patients who undergo
  successful resection (only 20 of patients) is
  approximately 12-19 months, with a 5-year
  survival rate of 15-20.
• Although discouraging, these results are still
  markedly better than those for patients with
  unresectable pancreatic carcinoma.

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Management

• The management of pancreatic carcinoma is a
  multidisciplinary process.
• Most patients initially present to their primary
  care practitioner with general symptoms such as
  abdominal pain, weight loss, or fatigue.
• Patients may also be seen initially by a
  gastroenterologist if they present with
  obstructive jaundice.
• Typically, the management of pancreatic cancer
  would entail consultations with a
  gastroenterologist, medical oncologist, general
  surgeon or surgical oncologist, and possibly a
  radiation oncologist.

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Benign Tumors of the Pancreas

• Insulinoma
• rare pancreatic tumor
• secretes insulin
• 10 cancerous
• Gastrinoma
• secretes above average levels of gastrin
• can cause peptic ulcers
• 50 cancerous
• Glucagonoma
• secretes glucagon
• Causes rash
• 80 cancerous

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Insulinoma

• An insulinoma is a rare type of pancreatic tumor
  that secretes insulin, a hormone that lowers the
  levels of sugar (glucose) in the blood.
• Only 10 of insulinomas are cancerous.

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Insulinoma

• Symptoms result from low levels of sugar in the
  blood.
• The symptoms include faintness, weakness,
  trembling, awareness of the heartbeat
  (palpitations), sweating, nervousness, and
  profound hunger.
• Other symptoms include headache, confusion,
  vision abnormalities, unsteadiness, and marked
  changes in personality.
• The low levels of sugar in the blood may even
  lead to a loss of consciousness, seizures, and
  coma.

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Insulinoma

• Very low levels of sugar and high levels of
  insulin in the blood can indicate the presence of
  an insulinoma.
• The location must be pinpointed. Imaging
  testssuch as CT, ultrasound, and arteriography
  of the intestinal arteriescan be used to locate
  the tumor, but sometimes exploratory surgery is
  needed

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Insulinoma

• The primary treatment for an insulinoma is
  surgical removal - cure rate of about 90.
• When the insulinoma cannot be completely removed
  and symptoms continue, several drugs (for
  example, streptozocin (Zanosar) and octreotide
  (Sandostatin)can be helpful

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GASTRINOMA

• A gastrinoma is a tumor usually in the pancreas
  or duodenum (the first segment of the small
  intestine) that produces excessive levels of the
  hormone gastrin, which stimulates the stomach to
  secrete acid and enzymes, causing peptic ulcers.

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GASTRINOMA

• Most people with gastrinomas have several tumors
  clustered in or near the pancreas.
• About half of the tumors are cancerous.
• Sometimes a gastrinoma occurs as part of multiple
  endocrine neoplasia, a hereditary disorder in
  which tumors arise from the cells of various
  endocrine glands, such as the insulin producing
  cells of the pancreas.

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GASTRINOMA

• The excess gastrin secreted by the gastrinoma
  causes Zollinger-Ellison syndrome (ZES)
• a rare disorder that causes tumors in the
  pancreas and duodenum and aggressive peptic
  ulcers in the stomach and duodenum

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Synonyms of Zollinger Ellison Syndrome

• Gastrinoma
• Pancreatic Ulcerogenic Tumor Syndrome
• Z-E Syndrome
• ZES

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GASTRINOMA

• However, as many as 25 of people with (ZES)
  Zollinger-Ellison syndrome may not have an ulcer
  when the diagnosis is made.
• Rupture, bleeding, and obstruction of the
  intestine can occur and are life threatening.

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GASTRINOMA

• For more than half of the people with a
  gastrinoma, symptoms are no worse than those
  experienced by people with ordinary peptic ulcer
  disease.
• In 25 to 40 of people, diarrhea is the first
  symptom.

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GASTRINOMA

• a gastrinoma is suspected when a person has
  frequent peptic ulcers or several peptic ulcers
  that do not respond to the usual ulcer
  treatments.
• Blood tests to detect abnormally high levels of
  gastrin are the most reliable diagnostic tests.

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GASTRINOMA

• High doses of proton pump inhibitors may be
  effective for reducing acid levels and relieving
  symptoms temporarily.
• About 20 of people who do not have multiple
  endocrine neoplasia can be cured with surgical
  removal of the gastrinoma.
• If these treatments fail, a total gastrectomy may
  be necessary.

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GASTRINOMA

• This operation does not remove the tumor, but the
  gastrin can no longer create ulcers after the
  acid-producing stomach is removed.
• If the stomach is removed, daily oral iron and
  calcium supplements and monthly injections of
  vitamin B12 are needed, because absorption of
  these nutrients is impaired when stomach juices
  that prepare these nutrients for absorption are
  no longer available.

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Gastrinoma
108
GASTRINOMA

• If cancerous tumors have spread to other parts of
  the body, chemotherapy may help reduce the number
  of tumor cells and the levels of gastrin in the
  blood.
• However, such therapy does not cure the cancer,
  which is ultimately fatal.

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Glucagonoma

• A glucagonoma is a tumor of the pancreas that
  produces the hormone glucagon, which raises the
  level of sugar (glucose) in the blood and
  produces a distinctive rash.

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Glucagonoma

• About 80 of glucagonomas are cancerous.
• However, they grow slowly, and many people
  survive for 15 years or more after the diagnosis.
  
• The average age at which symptoms begin is 50.
• About 80 of people with glucagonomas are women.

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Glucagonoma

• High levels of glucagon in the blood cause the
  symptoms of diabetes mellitus.
• Often, the person loses weight.
• In 90 of people, the most distinctive features
  are a chronic reddish brown skin rash (necrolytic
  migratory erythema) and a smooth, shiny, bright
  red-orange tongue.

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Glucagonoma

• The mouth also may have cracks at the corners.
• The rash, which causes scaling, starts in the
  groin and moves to the buttocks, forearms, and
  legs.

113
Glucagonoma syndrome -- necrolytic migratory
erythema
114
Glucagonoma

• The diagnosis is made by identifying high levels
  of glucagon in the blood and then locating the
  tumor by arteriography

115
Glucagonoma

• Ideally, the tumor is surgically removed, which
  eliminates all symptoms.
• However, if removal is not possible or if the
  tumor has spread, chemotherapy may reduce the
  levels of glucagon and lessen the symptoms.
• However, chemotherapy does not improve survival.

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Glucagonoma

• The drug octreotide also reduces glucagon levels,
  may clear up the rash, and may restore appetite,
  facilitating weight gain.
• Octreotide may elevate the levels of sugar in the
  blood even more.

117
Glucagonoma

• Zinc ointment may be used to treat the skin rash.
  
• Sometimes the rash is treated with intravenous
  amino acids or fatty acids.

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