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					 Diseases of the Pancreas
Victor Politi, M.D., Medical Director
SVCMC, School of Allied Health Professions,
Physician Assistant Program
           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).
             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.
 Accessory duct of Santorini
 Duct of Wirsung
             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.
           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.
          What is Pancreatitis?
   Pancreatitis is an inflammatory process in
    which pancreatic enzymes autodigest the
    gland
   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
   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.
   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.
 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.
    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
        Necrotizing pancreatitis
   When necrosis involves the parenchyma,
    accompanied by hemorrhage and
    dysfunction of the gland, the inflammation
    evolves into hemorrhagic or necrotizing
    pancreatitis
      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.
 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.
            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.
            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.
            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.
 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
                    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.
                    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.
                Physical
 Tachycardia
 Tachypnea
 Hypotension
 Fever
 Abdominal tenderness, distension,
  guarding, and rigidity
                 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.
                   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.
   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
                   Causes

   The major causes are long-standing
    alcohol consumption and biliary stone
    disease.
                    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
                 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
                  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.
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
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
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
Other problems to be considered
– Perforated viscus
– Acute peritonitis
– Choledocholithiasis
– Macroamylasemia
– Macrolipasemia
– Intestinal obstruction
– Pancreatic cancer
– Malabsorption syndromes/processes
    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.
    Acute Pancreatitis - Diagnosis
   Imaging Studies
    – X-ray
    – ultrasound
    – CT
                    Lab Studies
   A complete blood count (CBC) demonstrates leukocytosis
    (WBC >12000) 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
                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
               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
           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.
              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.
             Imaging Studies
   CT scan is the most reliable imaging
    modality in the diagnosis of acute
    pancreatitis.
Pancreatitis, Acute - CT Scan
Pancreatitis, Chronic - CT Scan
               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.
                 Treatment
   Most of the cases presenting to the ED are
    treated conservatively, and approximately
    80% respond to such treatment
                  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.
               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.
              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.
               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.
              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
                  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
                    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.
   Antibiotics
    – Ceftriaxone (Rocephin), Unasyn, Mefoxitin
    – Ampicillin (Marcillin, Omnipen),Gent, Flagyl
   Analgesics
    – Meperidine (Demerol)
                 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)
               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
                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
            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
               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.
Other Disorders of the
      Pancreas
         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
 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.
 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).
 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
 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%.
 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
   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.
 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.
        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.
         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.
                 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.
                   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.
                    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.
                      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.
                 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.
                  Physical
   Patients presenting with end-stage disease
    may have ascites, a palpable abdominal
    mass, hepatomegaly from liver
    metastases, or splenomegaly from portal
    vein obstruction.
                    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).
   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
   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.
                 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.
               Treatment
 Other therapies for pancreatic cancer
  should include palliation of the major
  symptoms of disease.
 Chemotherapy
 Radiation therapy
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
           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.
                 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.
                   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.
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
                 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.
                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.
               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
  tests—such as CT, ultrasound, and
  arteriography of the intestinal arteries—
  can be used to locate the tumor, but
  sometimes exploratory surgery is needed
              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
              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.
             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.
               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
    Synonyms of Zollinger Ellison
           Syndrome
 Gastrinoma
 Pancreatic Ulcerogenic Tumor Syndrome
 Z-E Syndrome
 ZES
            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.
            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.
            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.
            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.
              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.
Gastrinoma
            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.
               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.
             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.
             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.
             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.
 Glucagonoma syndrome --
necrolytic migratory erythema
               Glucagonoma
   The diagnosis is made by identifying high
    levels of glucagon in the blood and then
    locating the tumor by arteriography
            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.
             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.
             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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