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Definitions

Acute fluid collection. This occurs early in the course


of acute pancreatitis and is located in or near the
pancreas. The wall encompassing the collection is ill
defined.

Acute pseudocyst. A collection of pancreatic juice


enclosed in a wall of fibrous or granulation tissue
that arises followingan attack of acute pancreatitis.
Formation of a pseudocyst requires 4 weeks or
more from the onset of acute pancreatitis

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Definitions
Pancreatic necrosis. A diffuse or focal area of
nonviable parenchyma which is typically associated
with peri pancreatic
Pancreatic abscess. A circumscribed intraabdominal collection of pus, usually in proximity to
the pancreas containing little or no pancreatic
necrosis.
Pancreatic effusion. An encapsulated collection of
fluid arising as a consequence of acute pancreatitis,
typically in the pleural cavity.
Pancreatic ascites. Chronic generalised peritoneal
enzyme-rich effusion usually associated with
pancreatic duct disruption.

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Chronic pancreatitis

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Epidemiology
In temperate areas alcohol misuse accounts for
most cases
It mainly affects men aged 4050 years
The quantity and duration of alcohol consumption
correlates with the development of chronic
pancreatitis.
8.2 cases per 100000 and a prevalence of 27.4per
100000 were found in the prospective Copenhagen
Pancreatitis Study

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Nomenclature and definition


Chronic pancreatitis is a continuing inflammatory
disease of the pancreas that typically presents with
upper abdominal and back pain.
In 10 to 15per cent of patients, pain may be absent.
Only 15 to 20per cent have exocrine insufficiency
and a similar proportion are diabetic.
The only sign of an inflammatory process may be
fibrosis, indicative of earlier inflammation.

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Pathogenesis
Alcohol induced chronic pancreatitis - 150200 mg
alcohol/day - 3 to 7years
Increasing fibrosis of the acinar tissue and the
relative sparing of the islets until a late stage.
The viscosity of pancreatic secretion increases, with
protein precipitates developing; in addition, the
fibrotic process tends to form strictures of the main
and major ducts.

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Pathogenesis
Stone formation - nucleus of protein in
which nickel and iron can be found;
around this, layers of calcium
carbonate develop
The chronic pancreatitis that develops
in children and young adults in India
and other areas nearer the equator has
been labelled tropical chronic
pancreatitis
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Pathogenesis
The role of antioxidants in the
protective mechanisms is probably very
important and cigarette smoking has
an additional adverse a
20 cigarettes per day, irrespective of
alcohol intake, have a threefold
increase in the risk for developing
pancreatic cancerffect in those who
abuse alcohol.
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Computed tomogram showing dilated pancreatic duct


with
multiple calcified stones
P C Bornman, I J Beckingham
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Symptoms and signs


Severe dull epigastric pain radiating to the
back, which may be partly relieved by
leaning forward
Nausea and vomiting
Epigastric tenderness
Patients often avoid eating because it
precipitates pain. This leads to severe weight
loss, particularly if patients have
steatorrhoea.

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Symptoms and signs


Steatorrhoea presents as pale, loose,
offensive stools that are difficult to
flush away and, when severe, may
cause incontinence.
It occurs when over 90% of the
functioning exocrine tissue is
destroyed, resulting in low pancreatic
lipase activity, malabsorption of fat,
and excessive lipids in the stools.
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Symptoms and signs


One third of patients will develop overt
diabetes mellitus, which is usually mild
but brittle, with patients having a
tendency to develop hypoglycaemia
due to a lack of glucagon.
Hypoglycaemic coma is a common
cause of death in patients who
continue to drink or have had
pancreatic resection.
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Diagnosis
No reliable biochemical markers, and early
parenchymal and ductal morphological
changes may be hard to detect
Tests of pancreatic function are
cumbersome and seldom used to confirm
the diagnosis.
Early diagnosis is often made by exclusion
based on typical symptoms and a history of
alcohol misuse

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Diagnosis
In patients with more advanced disease:
enlarged and irregular pancreas, dilated
main pancreatic duct, intrapancreatic
cysts, and calcification. (X-Ray CT scan)
ERCP:
irregular dilatation of the pancreatic duct
with or without strictures, intrapancreatic
stones, filling of cysts, and smooth
common bile duct stricture
Endoscopic ultrasonography
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Complications

Pain
Obstruction of adjacent organs
Splenic venous thrombosis
Exocrine insufficiency
Endocrine insufficiency
Ascites or pleural effusion
Pancreatic pseudocyst
Pancreatic cancer

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Treatment
Management of acute attacks of pain and, in
the long term, control of pain and the
metabolic complications of diabetes mellitus
and fat malabsorption
The major indications for treatment are:
intractable pain;
fear of carcinoma;
the development of structural
complications

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Medical management
Analgesics
The mainstay of treatment remains abstinence
from alcohol
Enzymes for secretory insuficiency
It is thus important to undertreat rather than
overtreat diabetes in these patients, and they
should be referred to a diabetologist when early
symptoms develop.
Oral hypoglycaemic drugs should be used for as
long as possible.
Major pancreatic resection invariably results in
the development of insulin dependent diabetes.

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Endoscopic procedures
To remove pancreatic stone after ESWL
(few centers)
To stent strictures or pseudocyst

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Surgery
Only after all forms of conservative
treatment have been exhausted and
when it is clear that the patient is at
risk of becoming addicted to narcotics.
As much as possible of the normal
upper gastrointestinal anatomy and
pancreatic parenchyma should be
preserved to avoid problems with
diabetes mellitus and malabsorption of
fat.
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Surgical approaches
Interruption of nerve pathways
Laparoscopic or transthoracic
splanchnicectomy

Bypass and drainage


gastrojejunostomy and cholecysto- or
choledochojejunostomy
pancreatojejunostomy

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Resections
Duodenum-preserving, head-of-pancreas
resections (Beger, Frey)
More extensive resections such as Whipple's are
occasionally required.
Beneficial outcome in 6070% of cases at five
years, but the benefits are often not sustainable
in the long term.
It is often difficult to determine whether failures
are surgically related or due to narcotic
addiction.

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Pancreatic cancer

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Pancreatic cancer
In USA the incidence rates for pancreatic cancer
increased nearly threefold from 1920 to 1978.
The annual pancreatic cancer incidence rate has
remained constant at - 9 new cases per 100 000
population,
In nearly all European countries, the incidence rates
of pancreatic cancer have continued to rise.
Throughout the world, over 185 000 new cases of
pancreatic cancer occur yearly, with a death-toincidence ratio of approximately 0.99.

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Ductal adenocarcinoma
Clinically, 80% of pancreatic tumours are
malignant ductal adenocarcinomas
It is generally true that the incidence rates of
pancreatic cancer are highest in Western and
industrialized countries, and lowest in
underdeveloped nations.
Pancreatic cancer is more common in the
elderly with most patients being >60 years
old

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Risk factors
Pancreatic cancer is more common in African
Americans and slightly more common in
men than women.
The risk of developing pancreatic cancer is
two to three times higher if a parent or
sibling had the disease.
Cigarette smoking. Smoking increases the
risk of developing pancreatic cancer by at
least twofold due to the carcinogens in
cigarette smoke
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Risk factors
Diabetes
Diets high in fat and low in fiber, fruits, and
vegetables are thought to be associated with
an increased risk of pancreatic cancer
Chronic pancreatitis, especially familial
pancreatitis, have an increased risk of
developing pancreatic cancer
It is estimated that up to 10% of pancreatic
cancers occur as a result of an inherited
genetic predisposition - familial adenomatous
polyposis (FAP), von Hippel-Lindau disease
(VHL), hereditary breast cancer
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Von Hippel-Lindau
Von Hippel-Lindau
syndrome (VHL) is a
hereditary condition
associated with
hemangioblastomas
(blood vessel
tumors) of the brain,
spinal cord, and eye.

Slit lamp photograph showing retinal


detachment in Von Hippel-Lindau
disease.

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Pathology: Cell Types


3 different epithelial cell types
Acinar cells (80% by gland volume)
Ductal cells (10-15%)
Endocrine (islet) cells (1-2%)

95% of malignant pancreatic


neoplasms arise from acinar and ductal
cells

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Diagnosis
Dull midepigastric pain, malaise, nausea, fatigue,
and weight loss.
Two thirds of pancreatic cancers develop in the
head of the pancreas, and most patients present
with progressive, obstructive jaundice with dark
urine and pale stools - painless jaundice
Pruritus
Severe cachexia, as a result of increased energy
expenditure mediated by the tumour, is also a poor
prognostic indicator.
Cachexia is the usual presenting symptom in
patients with tumours of the body or tail of the
pancreas.

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Diagnosis
New-onset diabetes within the year
prior to diagnosis is found in 15% of
patients with pancreatic cancer
Courvoisier sign (painless jaundice
with a palpable gallbladder) is an
associated finding in patients with
periampullary tumors and primary bile
duct tumors.
Trousseau's sign (migratory
thrombophlebitis) has been associated
with pancreas cancer.
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Laboratory tests
Elevated serum bilirubin.
Elevated alkaline phosphatase.
Mild elevations of serum glutamicoxaloacetic transaminase and serum
glutamate pyruvate transaminase.
Tumor markers:
CA19-9
CEA

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Imaging
US is not as sensitive as CT and is limited by
the presence of bowel gas.
CT-scan
MRI may provide as much information as does
CT but is costly, is limited in availability, and
takes longer
Endoscopic ultrasonography can assess tumor
size as well as portal and mesenteric vascular
involvement and obtain tissue samples.
Staging laparoscopy may be useful in
identifying small liver metastasis and
peritoneal implants.
Positron emission tomography (PET) is limited
to investigating for occult metastatic disease
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CT-scan
Hypoattenuating indistinct mass that
distorts the normal architecture of the gland,
often paired with findings of a dilated
pancreatic or biliary ductal system (the socalled double-duct sign)
The CT criteria used to define potential
resectability are as follows:
Absence of distant metastasis.
Preserved fat plane between the mass and the
superior mesenteric vein (SMV)/portal vein (PV).
Absence of tumor encasement of the hepatic,
celiac, or superior mesenteric arteries.
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Double duct sign in 47-year-old man with ampullary carcinoma.

Ahualli J Radiology 2007;244:314-315


2007 by Radiological Society of North America

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Staging - Tumor

TX: Primary tumor cannot be assessed


T0: No evidence of primary tumor
Tis: Carcinoma in situ
T1: Tumor is limited to the pancreas and is 2 cm
in greatest dimension
T2: Tumor is limited to the pancreas and is >2 cm
in greatest dimension
T3: Tumor extends beyond the pancreas but
without involvement of the celiac axis or the
superior mesenteric
artery
T4: Tumor involves the celiac axis or the superior
mesenteric artery (unresectable primary tumor)

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Staging Nodes and


NX: Regional lymph nodes cannot be
assessed
N0: No regional lymph node metastasis
N1: Regional lymph node metastasis
Distant metastasis (M)
MX: Distant metastasis cannot be
assessed
M0: No distant metastasis
M1: Distant metastasis
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Staging

Stage
Stage
Stage
Stage
Stage
Stage
Stage

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0 - Tis, N0, M0
IA - T1, N0, M0
IB - T2, N0, M0
IIA - T3, N0, M0
IIB - T1-3, N1, M0
III - T4, Any N, M0
IV - Any T, Any N, M1

Prognosis
Timing of disease-associated symptoms
Median survival: 8-12 months for locally
advanced unresectable disease, and 3-6
months for those who present with
metastases

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Investigation and management of pancreatic ductal


carcinoma
I J BECKINGHAM - ABC OF LIVER, PANCREAS AND GALL
BLADDER

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Curative resection
Pancreaticoduodenectomy (Whipple procedure)
consists of en bloc resection of the head of the
pancreas, distal common bile duct, duodenum,
jejunum, and gastric antrum.
Pylorus-sparing pancreaticoduodenectomy
(alternative)
Distal pancreatectomy for body and tail tumors
Extended lymphadenectomy, including nodes from
the celiac axis to the iliac bifurcation and nodes
from the portal vein and superior mesenteric
artery, has not been shown to affect survival but
does increase morbidity
Mortality of less than 5% in specialized centers.
Five year survival rate of 1015%
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Otto van Delden and Robin Smithuis

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PA Clavien et al

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PA Clavien et al

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PA Clavien et al

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PA Clavien et al

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PA Clavien et al

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Curative resection
Only 15 to 20% of patients with
pancreatic cancer have tumors that are
resectable for cure at the time of
presentation, because either metastatic
disease or extensive local involvement
precludes resection.
Very few tumours of the body and tail
are resectable (3%) as patients usually
present late with poorly defined
symptoms.
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Adjuvant therapy
Radiotherapy and chemotherapy
5-FU

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Palliation
The goals are to offer prevention or
relief from the three major problems
that present in advanced metastatic
pancreas cancer: (1) obstructive
jaundice, (2) gastric outlet obstruction,
and (3) pain.

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Nonoperative Palliation
Biliary decompression can be achieved
by either endoscopic or percutaneous
transhepatic techniques in nearly all
patients who are not candidates for
surgical intervention.
Pain treatment (includes CT-guided
celiac block)

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Metal wall stent in common bile duct of patient with


pancreatic carcinoma. (Note contrast in gall bladder from
endoscopic retrograde cholangiopancreatography)

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Surgical palliation
For obstructive jaundice and gastric
outlet obstruction is achieved by
performing a biliary internal drainage
procedure such as
choledochojejunostomy and
gastrojejunostomy.
Splanchnicectomy

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Cystic tumours
1% of all pancreatic neoplasms
The mucinous type (about 50%) is
premalignant

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Cystic tumours
Serous cystadenoma are benign lesions and are
usually asymptomatic
Mucinous cystic neoplasms are considered
premalignant lesions and account for 35% of all
CPNs.
Intraductal papillary mucinous tumors (IPMTs)
account for 25% of all CPNs and have a slight male
predominance. IPMTs carry a very high malignant
potential, up to 40%
Other rare cystic pancreatic neoplasms (remaining
10%) include cystadenocarcinoma, acinar cell
cystadenocarcinoma, cystic choriocarcinoma, cystic
teratoma, and angiomatous neoplasms.
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Endocrine tumours
Tumor Name Major Hormone(s)

Syndromes

Insulinoma

Insulin

Hypoglycemic

Gastrinoma

Gastrin

Ulcerogenic

Zollinger-Ellison
syndrome

VIPoma

Vasoactive intestinal
polypeptide

Watery diarrhea,
hypokalemia,
achlorhydria

Verner-Morrison
syndrome

Glucagonoma Glucagon cutaneous

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Hyperglycemic

Eponym

Insulinoma
About 75% are solitary and benign.
About 10% are malignant, and
metastases are usually evident at the
time of diagnosis.
The remaining 15% are manifestations
of multifocal pancreatic disease

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Clinical symptoms
The symptoms (related to cerebral
glucose deprivation) are bizarre
behavior, memory lapse, or
unconsciousness.
Hypoglycemic episodes are usually
precipitated by fasting and are relieved
by food, so weight gain is common

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Whipple's triad
Is present in most cases:
(1) hypoglycemic symptoms produced by
fasting,
(2) blood glucose below 50 mg/dL during
symptomatic episodes, and
(3) relief of symptoms by intravenous
administration of glucose.

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Imaging
Angiography gives a yield of about
50%.
Transhepatic portal venous sampling
has proved an accurate preoperative
localizing method, demonstrating the
position in the pancreas in about 95%
of lesions.

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Treatment
In about 10% of cases, the tumor is so small
or located so deeply that it is difficult or
impossible to find at laparotomy.
High-resolution CT and MR scans are
successful in demonstrating about 40% of
tumors.
Endoscopic (gastroscopic) ultrasound
examination of the pancreas may be able to
show a much higher percentage.
The most important examination is
intraoperative ultrasound, which can identify
a pancreatic tumor in nearly all cases.
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Medical treatment
Diazoxide is administered to suppress
insulin release.
For incurable islet cell carcinomas,
streptozocin is the best
chemotherapeutic agent.

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Surgical Treatment
Patients with insulinoma associated
with MEN-1 usually have multiple
(average of three) lesions.
Enucleation
Resection

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