Transcript Diseases of the pancreas

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.
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, lifethreatening 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
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 glutamicoxaloacetic 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 grambacilli 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

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.
