PATHOLOGY OF THE LIVER, BILIARY TRACT AND EXOCRINE
PANCREAS.
I.
CIRCULATORY DISORDERS
-hepatic chronic passive congestion- very common- results from
chronic right-sided heart failure
-central hemorrhagic necrosis- less common, in severe right-sided
heart failure- perivenous areas are more susceptible to ischemia than the
periportal tracts
-cardiac sclerosis-cirrhosis- rare- in long-standing chronic passive
congestion, increased amount of collagen fibres, associated with mild
regeneration
-infarcts- rare- because of double blood supply from hepatic
arteries and portal veins occur when an intrahepatic branch of hepatic
artery is occluded-in polyarteriitis nodosa, in thrombosis induced by
inflammation, in embolism
-Budd-Chiari syndrome- is thrombosis of the major hepatic veins
and often of the adjacent part of the vena cava
known possible causes of the Budd-Chiari syndrome include
-hematologic disorders with high tendency to develop thrombosispolycythemia vera
-use of contraceptive,
-tumors- such as hepatocellular carcinoma and renal cell ca- both
have tendency to grow within the veins,
-intrahepatic infections
idiopathic- very often without any apparent cause (1/3 cases)
consequencies: ascites, swollen liver, portal hypertension, esophageal
varices, death within months due to hepatic failure
-portal vein thrombosis- results in portal hypertension, ascites,
esophageal varices major causes of portal thrombosis- are abdominal
infections leading to portal vein phlebitis
-cancer arising in hepatic or extrahepatic sites and invading the
veins
-portal vein thrombosis may be due to propagation of the splenic
vein thrombi, such as in pancreatitis
- may occur after abdominal surgery or in liver cirrhosis
-so-called venoocclusive disease -clinically very important
condition
1
is defined as endothelial thickening, sclerosis and even occlusion of
multiple small and central veins possible causes include:
-graft-versus-host disease- this reaction may even clinically look
like Budd-Chiari syndrome
-may occur as a consequence of drug administration, including some
anti-cancer agents or may be caused by radiation
PEDIATRIC LIVER DISEASES.
-Neonatal hepatitis- many different diseases are included in the
term neonatal hepatitis, most of them- idiopathic
the rest- caused by various agents, like viruses, bacteria, treponema
pallidum, by metabolic disorders, such as alfa-1-antitrypsin deficiency,
some of these causes are not even infectiveclinically: most patients are male newborns, immature with low birth
weight
micro: lymphocytic infiltrates in the portal tracts, increased number of
Kupfer cells, presence of bile in ductules-cholestasis, disorder of normal
architecture of the liver and giant hepatocytes
-Extrahepatic biliary atresia- complete obstruction of at least
some of the hepatic bile ducts or common bile duct
clinically: infants develop progressive jaundice early after birth
usually full-term female infants
micro: cholestasis, inflammatory and fibrous reaction around
intrahepatic bile ductules, proliferation of aberrant marginal
ducts, periportal fibrosis and cirrhosis
surgical procedures may sometimes help to correct the defect- or liver
transplantation
-Reye’s syndrome- is a rare disease characterized by fatty
change in the liver and encephalopathy- often fatal
affects young children
-typically it develops following a viral infection or drug administration,
pathogenesis is unknown
pathology: diffuse fatty change of the liver
severe brain edema- swollen astrocytes
kidney- slight fatty change
jaundice is absent
INFECTIONS OF THE LIVER.
-Viral hepatitis.
pathologically- severe regressive changes of hepatocytes including
necrosis and presence of inflammation
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clinically-malaise, fever, jaundice and laboratory evidence of liver cell
necrosis (elevated serum levels of transaminases)
it presents as acute self-limited disease, less commonly it may persist
and cause chronic inflammation resulting in fibrosis and even progressive
to cirrhosis
caused by hepatotropic viruses- affect primarily hepatocytes, other
infective agents, such as cytomegalovirus, tuberculosis, infectious
mononucleosis-with primary infections of other organs-rarely
encountered
hepatotropic viruses-include hepatitis A virus, hepatitis B virus, B
hepatitis-associated delta virus, hepatitis C virus and non-A non-B
hepatitis virus
Hepatitis A virus -RNA virus, causes a benign, acute self-limited
disorder that does not lead to chronic infection
previously called- infectious hepatitis -short-incubation hepatitis
(incubation period is from 15 to 45 days)
-transmission exclusively by fecal-oral route -poor hygiene, close
contacts-often as epidemic infections
-viremia is very short, thus blood-borne transmission is rare
Hepatitis B virus- previously known as serum hepatitis- long-incubation
hepatitis (incubation period from 30 days to 6 months)-DNA virus- Dane
particles are complete virions
three well defined antigens are associated with hepatitis-B virus : two
are associated with the virus core (HBcAg and HBeAg) and the third with
the outer surface coat (HBsAg)
antibodies to these antigens can be used as markers for determining the
clinical status of a patient
-blood and body fluids of infected persons are the most important
source for infection- HBV is most often transmitted by the parenteral
route- blood transfusions, infusion of plasma and other blood fragments,
by dental and surgical instruments, hemodialysis, autopsy, transplantation
clinical spectrum of B-hepatitis is broader- acute self-limited disorder,
but the infection may result in chronic hepatitis, chronic carrier state, or
cirrhosis
pathogenesis:
there are two major mechanisms of liver injury in viral hepatitis
-direct cytopathic effect
-induction of immune response against viral antigens and damage of
virus-infected hepatocytes thus rapid immune reaction in acute viral
hepatatis may in some cases cause cellular injury and eliminate the virus
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-fulminant viral hepatitis-associated with liver necrosis and total
elimination of the virus- no carrier state, no chronic hepatitis and
cirrhosis
-total failure of immune response accompanied by mild liver injury
but persistent viremia
Clinical syndromes in viral hepatitis:
1.- Asymptomatic infection- only detected antibodies
2.- Acute hepatitis icteric or anicteric
3.- Fulminant hepatitis with massive hepatic necrosis
4.- Carrier state
5.- Chronic hepatitis- persistent or active
Acute viral hepatitis
can be caused by all hepatotropic viruses
-is either icteric or anicteric,
- usually pruritus, jaundice is due to elevation of predominantly
conjugated bilirubin-dark urine due to presence of urobilinogen
histological findings:
-focal regressive changes including necroses of hepatocytesballooning degeneration of hepatocytes, prominent in centrolobar areas,
death of individual hepatocytes often by apoptosis ( so-called Councilmans
bodies)
-reactive and inflammatory changes- activation of Kupfers cells,
hyperplasia of portal macrophages, portal inflammatory infiltrate
composed mostly of hepatocytes, acute inflammatory cells around dead
hepatocytes-resorptive granulomas
-bile stasis
-evidence of regeneration of hepatocytes- enlarged nuclei,
binucleated hepatocytes- regeneration may be complete but not always
Fulminant viral hepatitis
very rare manifestation of viral infection- in about 1-4% of patients with
HBV
the onset is very rapid- hepatic failure- with hepatic encephalopathy
pathology: massive or submassive liver necrosis-necrotic areas may be
partly healed-by fibrosis, coagulative to liquefactive necrosis, little
inflammmatory reaction- if the patient survives-irregular nodular
regeneration- little or no residual scarring in the liver -overall poor
prognosis, if survived no tendency to become carrier, no propensity to
develop cirrhosis
Chronic hepatitis
is defined as the continuation of hepatic inflammation and necrosis for
longer than six months
4
in majority of cases- present with persistent elevated levels of liver
enzymes, such as aminotranferases and alkaline phosphatase, usually no
other symptoms, but some patients may experience episodes of malaise,
loss of appetite, nausea, mild jaundice traditionally two forms are
distinguished
chronic persistent hepatitis- minimal necrosis of hepatocytes,
lobular architecture is preserved, portal tracts are inflammed but well
circumscribed
the course is benign
chronic active hepatitis- more progressive liver destruction
micro: severe portal and periportal infiltrates of lymphocytes, plasma
cells and macrophages, active destruction of hepatocytes in the vicinity
of portal tracts- so-called piecemeal necroses, fibrosis and cirrhosis it is
getting to be evident now that both forms are related may overlap
Autoimmune chronic hepatitis
is a chronic hepatitis without relation to viral infection, female
preponderance, elevated serum IgG levels and presence of anti-nuclear
antibodies (ANCA) and anti-smooth muscle antibodies (ASMA)
sometimes- association with Sjogrens syndrome, responds well to the
administration of steroids
IV. ALCOHOLIC LIVER DISEASE
chronic abuse of alcohol may produce three patterns of liver injury
-fatty livermost common, fully reversible, the fatty change affects mostly
centroacinar hepatocytes- the cells demonstrate macrovesicular
and typically microvesicular fatty change
-alcoholic hepatitis
after heavy drinking,
- histologically it is characterized by swelling and necrosis of
hepatocytes, inflammatory reaction around foci of necrosis, presence of
cytoplasmic hyaline (Mallory bodies) within the affected liver cellsstructures are derived from cytoskeletal intermediate filaments of
cytokeratin and vimentin type, there may be intrahepatic cholestasis
-alcoholic cirrhosis
this is a final and irreversible form of alcoholic liver disease
-liver is first enlarged, but in most cases the liver is shrunken with lower
weight, micronodular cirrhosis is typical
microscopically: fibrous septa interconnecting portal areas and bridging
portal tracts with central veins, scarring and regeneration, total
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effacement of previous structure, reactive bile duct proliferation at
margins of distended portal tracts,lymphocytic infiltrates
Clinical consequencies of fully developed alcoholic cirrhosis are same as in
postnecrotic c.
V.
POSTNECROTIC CIRRHOSIS
the term postnecrotic cirrhosis is applied to all types of cirrhosis not
associatied with chronic alcohol abuse, not biliary origin
morphology:
grossly:liver is of normal size or enlarged, or slightly shrunken, hallmark
is nodular structure- nodules are typically large- macronodular cirrhosis
micro: broad scars and disordered islands of liver parenchyma, within the
scars there is a heavy inflammatory infiltrate (lymphocytes) and
proliferating marginal bile ducts,
-the more necrotic hepatocytes- the more active cirrhosis
prognosis is very hard to predict
clinical manifestation of cirrhosis:
-relate to portal hypertension:
grossly - distended abdomen filled with ascites fluid, spider
angiomas in the skin, splenomegaly and portocaval shunts, most importantesophageal varices
-relate to destruction of bile ducts -jaundice
hepatic encephalopathy and hepatic coma
most common causes of death include hepatic failure, GI bleeding
commonly from esophageal varices, and hepatorenal syndrome
INTRAHEPATIC BILIARY TRACT DISEASES.
BILIARY CIRRHOSIS- three major causes of biliary cirrhosis
1.-primary biliary cirrhosis
2.-primary sclerosis cholangitis
3.-secondary biliary cirrhosis
1.- Primary biliary cirrhosis (PBC)
-chronic progressive cholestatic liver disease characterized by
destruction of intrahepatic bile ducts, portal inflammation and scarring,
and sometimes by cirrhosis and hepatic failure
pathogenesis: immune-mediated disease- serum is positive for
antimitochondrial antibodies - in 90% of patients- in some patients other
autoimmune disorders, such as Hashimoto thyroiditis, Sjogrens syndrome
-primarily a disease of middle-aged women
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clinical symptoms: onset is slow, with pruritus, hepatomegaly, jaundice and
xanthomas ( elevated serum and tissue cholesterol)
pathology:
first stage- destructive inflammatory lesions of multiple
interlobar and septal small bile ducts characterized by granulomatous
inflammation accompanied by dense mixed infiltrate in portal tracts
progressive lesion- global involvement of hepatic portal
tracts with secondary obstructive changes and eventually with
development of cirrhosis
end stage- indistinguishable from other forms of cirrhosis
2.-primary sclerosing cholangitis
-rare disorder affecting various segments of extrahepatic and
intrahepatic biliary tract -chronic progressive cholestatic liver disease characterized by
inflammation, obliterative fibrosis and segmental dilatation of the intraand extrahepatic bile ducts
-commonly seen in association with inflammatory bowel disease (in
up to 70 % of cases), particularly with chronic idiopathic ulcerative colitis
more common in middle-aged men, pathogenesis is unknown- antibodies
are usually absent
morphology: periductal fibrosis with mild inflammation- typically
prominent in large ducts
3.- Secondary biliary cirrhosis - chronic progressive cholestatic
liver disease characterized by obliteration of intrahepatic bile ducts
most common causes include:
-it is encountered in patients with bile stones (cholelithiasis), strictures
for example from previous surgical procedures, cancers of extrahepatic
biliary tree and of the head of pancreas,etc
-obstruction results in increase of bile pressure within extrahepatic
biliary ducts and intrahepatic bile tree - cholestasis may be severe (is
reversible) -periportal fibrosis- leads to cirrhosis (irreversible)
- end stage liver is yellow-green and finely divided by fibrous septa, small
and large bile ducts are distended and contain inspisated bile
-incomplete obstruction is always associated with risk of infection bacterial suppurative infection (cholangiolitis)- infiltration of bile ducts
and portal tracts by leukocytes, and abscess formation - biliary sepsis
Clinical findings in biliary cirrhosis- variable, prominent jaundice and
pruritus are common in all forms, portal hypertension is uncommon
endoscopic retrograde cholangiopancreatography (ERCP)- clue to
diagnosis
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ANOMALIES OF THE BILIARY TREE
altered architecture of the intrahepatic biliary tree
-von Meyenburg complexes- small clusters of dilated bile ducts in
fibrous stroma
-an incidental portal tract finding
-polycystic liver disease- characterized by presence of few to
hundreds of biliary epithelium-lined cystic lesions 1-5 cm in diameter,
-seen in association with polycystic kidney disease
-congenital hepatic fibrosis- incomplete involution of embryonic
ductal structures, liver is subdivided by dense fibrous septa with
embedded, irregular biliary structures
-portal hypertension and esophageal varices are common
-Caroli disease- segmental dilatation of larger ducts of
intrahepatic biliary tree, complicated by cholelithiasis hepatic abscesses
and cholangiocarcinoma
METABOLIC LIVER DISEASES
Hemochromatosis-pigment cirrhosis
-is an uncommon disorder characterized by accumulation of iron within
the body due to increased poorly controlled GI uptake of iron- defect lies
at the level of enzymes in mucosal cells in the duodenum where iron is
absorbed from food
-hereditary autosomal recessive disorder
pathology: iron accumulates as ferritin and hemosiderin in parenchymal
tissues (liver, pancreas, myocardium, endocrine glands)
-iron deposition in the liver is toxic for hepatocytes- mechanism of
this toxicity is unclear-slowly developing cirrhosis and heavy deposits of
hemosiderin
1- liver: first histologically normal, later becomes progressively fibrotic
and heavily pigmented - progresses to micronodular cirrhosis
2pancreas: diffuse interstitial fibrosis with atrophy-results in
diabetes mellitus
3- skin: pigmentation of the skin is due to deposition of hemosiderin in
dermal macrophages and fibroblasts
other organs: diffuse myocardial fibrosis may lead to cardiomyopathy,
testes become atrophic, joint synovialitis- dues to depostis of
hemosiderin
Wilsons disease
-is an autosomal recessive disorder of copper metabolism in which liver
disease is a major component
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copper is deposited in many organs, such as liver, brain, and eyehepatolenticular degeneration
liver: hepatocytes accumulate copper- initially it was postulated that a
major cause is lack of ceruloplasmin- now it is believed the the problem is
in defect of lysosomal degradation of ceruloplasmin-copper complexes in
hepatocytes
- low ceruloplasmin levels in serum
-changes in liver include hepatitis, chronic or active, rarely fulminant
necrosis, often cirrhosis
brain: toxic injury affect the basal ganglia- atrophy of putamen
eye lesion: Kayser-Fleischer rings- green to gold deposits of copper in the
limbus of the cornea
alfa-1-antitrypsin deficiency
is a genetic autosomal recessive disorder characterized by abnormally low
serum levels of major protease inhibitor - alfa-1 antitrypsin
-that may lead to pulmonary emphysema and hepatic injury
the most common form of liver injury in alfa-1-AT deficiency is neonatal
hepatitis- rare generally, but relatively common cause of cirrhosis in
children
liver disease in adults- chronic hepatitis or full-blown cirrhosis
TUMORS OF THE LIVER
benign primary tumors and pseudotumorous lesions of the liver:
1. cavernous hemangioma
-most common primary liver tumor, occurs at all ages, most tumors remain
undetected, only those over 5 cm in diameter are likely to cause clinical
symptoms (palpable mass, episodes of pain due to thrombosis and
infarctions only in minority of cases)
grossly -benign, well circumscribed, most are subcapsular, reddish-blue in
colour
histologically: it consists of well differentiated vascular channels lined by
endothelium, may show thrombosis,scarring, hyalinization, and
calcification
2. Infantile hemangioendothelioma
-this is rare tumor, it is however the commonest mesenchymal tumor of
the liver in childhood
clinical symptoms include hepatomegaly and cardiac failure due to
arteriovenous shunts within the tumor, thrombocytopenia, anemia, and
hypofibrinogenemia may develop
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-these tumors are ill-defined, locally aggressive, may be solitary or
multiple, macroscopically- spongy, redbrown, and often fibrous in the
centre
microscopically- they are made up of communicating vascular channels
lined by endothelial cells which form multiple layers or tufts, the amount
of supporting stroma is variable, tratment is possible only if the tumor is
solitary
3. focal nodular hyperplasia
grossly- well circumscribed demarcated unencapsulated solitary or
multiple nodules in the liver- with absence of cirrhosis - white colour
microscopically- composed of central fibrous stellate-shaped scar
surrounded by normal liver structures
-most likely hamartoma and not true neoplasm, occurs in young and
middle-aged adults
4. hepatocellular adenoma
-rare benign tumor up to 30 cm in diameter, occurs in young women,
strongly linked to chronic use of oral contraceptives,
-liver cell adenoma occurs in normal liver, it is usually single and large (up
to 15 cm in diameter), well-defined but not encapsulated
histologically: composed of sheets and cords well differentiated
neoplastic hepatocytes with arteries and veins, portal tracts with bile
ducts are absent
malignant tumors:
1. hepatocellular carcinoma
-shows a remarkable geographical variability-the highest rates are in
Africa (Mosambique), South-East Asia, and Taiwan- these differnces are
best explained by different hepatitis B carries rates in different areas
-in 60 to 80% of patients liver cell carcinoma arises in cirrhotic liver
-the risk of cancer is particularly high in postnecrotic cirrhosis in HBV
infection- strong causal relationship has been established between
hepatotropic viral infection and liver cell carcinoma
grossly: massive huge tumor produces hepatomegaly, or it presents as
multiple nodules throughout the liver, or diffuse infiltration, foci of
hemorrhage and necroses are common, the tumor shows high propensity
to invade vessels, may obstruct hepatic veins- which results in BuddChiari syndrome or may obstruct portal vein- leads to portal hypertension
histologically: range from well-differentiated to highly anaplastic
undifferentiated tumors
well-differentiatedhepatocytes
arranged
in
trabecular
(sinusoidal) or acinar (tubular) patterns
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poorly differentiated- markedly pleomorphic giant cells, small
completely undifferentiated cells, spindle cells, or completely anaplastic
cells- hepatocellular features include only formation of bile
2. fibrolamellar carcinoma
is a distinctive variant of hepatocellular carcinoma with better prognosis
- occurs in noncirrhotic liver in young adults and children
histologically: single often encapsulated mass composed of eosinophilic
polygonal hepatocytes with PAS positive cytoplasm and cytoplasmic
hyaline globules, and of abundant thick bundles of collagenous stroma
3. hepatoblastoma
-this is rare malignant tumor of the liver, occurs in childhood, the usual
presentation is progressive enlargement of the abdomen due to
hepatomegaly, loss of weight, fever, vomiting and diarrhoe
macro- the tumor shows hemorrhages, cystic degeneration, necrosis,
histologically- the tumor consists of immature liver cells arranegd in solid
foci, trabeculae and primitive glandular structures with variable amounts
of primitive mesenchymal component
-rapidly progressive, kills the patient by liver failure, or metastases
4. cholangiocarcinoma
-arises from epithelial elements of the intrahepatic biliary tree
grossly: cirrhosis is not present, may appear as a unifocal large mass,
multifocal or diffusely infiltrative tumor
-unlike hepatocellular carcinoma- tumor is typically pale- since biliary
epithelium does not produce bile, firm in consistency- typically
desmoplasia (is a production of abundant tumor stroma)
histologically: more or less differentiated adenocarcinoma, often with
some mucus production,
prognosis: is poor, most patients die in 4-6 months, metastases develop
by lymphatics, and blood (lung)
5. epithelioid hemangioendothelioma
-the tumor has only recently been recognized, average age of the
patients is 50,
-presentation is nonspecific- weight loss, upper abdominal discomfort,
jaundice is rare
-the tumor is usually multiple, white and firm in consistency
histologically-the cells are epitheloioid - abundant cytoplasm, vesicular
nuclei, they appear to grow in irregular clumps, or sinusoids, some cells
display intracytoplasmic slit-like lumina with or withour ery, factor VIIIrelated antigen is positive
prognosis: growth is slow, most patients survive for 5 years
6. hepatic angiosarcoma
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extremely rare, highly aggressive malignant tumor, clear association to
occupational exposure to vinyl chloride and arsenic
7. the most common hepatic neoplasms are metastatic carcinomas
most common primary sites include large bowel, lung, and breast, but all
malignant tumors may give rise to meta in the liver
macro- multiple nodules, often replacing large parts of the liversurprisingly little abnormalities in function of the liver
PATHOLOGY OF BILIARY TRACT
diseases of biliary tree are very commoncholelithiasis- gallstones
cholecystitis
tumors of biliary tract
-these three pathologic conditions account for more than 90% of
diseases of biliary tract
-cholelithiasis
is presence of gallstones in the gallbladder or biliary tree-very common,
more frequent in women, peak incidence 4th decade
risk factors: obesity, female preponderance probably relate to estrogens
(higher incidence by use of oral contraceptives, higher risk in multiparous
women), for cholesterol gallstones-ethnic or genetic predisposition
 -acute cholecystitis
-majority of cases of cholecystitis are associated with stones (acute
calculous cholecystitis)- exact mechanism of inflammation is unknownmultifactorial (infection, mechanical irritation)
-acute acalculous cholecystitis-is difficult to explain- its incidence is
higher in critically ill adults (recent surgery, burns, trauma,etc)
morphology: the gallbladder is enlarged, tense, edematous, red, covered
by purulent exudate- sometimes gangrene or perforation
micro: the wall of the gallbladder is thickened, extensive inflammatory
ulceration of the mucosa , mixed inlfammatory infiltration- leukocytes
if the lumen is filled by pus- empyema of the gallbladder
 chronic cholecystitis
due to repeated attacks of acute inflammation, but in most cases it
develops without previous history of acute cholecystitis,
-almost always associated with gallstones
-pathogenesis: persaturation of bile predispose both to inflammation and
stones-role of bacteria is dubious
morphology: gallbladder may be enlarged, but more often it is contracted,
the wall is whitish-gray, serosa smooth, glistening, mucosal ulcers are
rare, inflammatory infiltrattion is focal and consist of lymphocytes
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-when stone is impacted in the neck of the gallbladder or cystic duct for
longer period-resorption of bile may occur-leaving clear mucinous
secretion- this is called hydrops of the gallbladder -chronic cholecystitis
may result in excessive fibrotization, hyaline change and dystrophic
calcification of the wall of the gallbladder- porcelain gallbladder
Clinical course: gallstones are silent (in approximately 70%) or provoke
severe clinical symptoms, such as
-induction of chronic cholecystitis
-give rise to obstruction of cystic or common bile duct manifesting
as attack of pain (biliary colic)
-predispose to acute pancreatitis
-may play a role in development of carcinoma of the gallbladder
-predispose to suppurative acute cholangitis and biliary sepsis
Carcinoma of the gallbladder
is the most common tumor of the biliary tract
in most cases- gallstones are present, more often in women, peak age in
7th decade
histologically: most cases are adenocarcinomas, about 10% are
adenoacanthomas or adenosquamous carcinomas
grossly: either an infiltrative pattern results in thickening of the whole
gallbladder wall or exophytic lesion fungating into the lumen -carcinoma
spreads locally aggressively,
clinical symptoms: obstruction of common bile duct- jaundice
or spread to porta hepatis, and lymph nodes and into the liver
very often secondary suppurative cholangitis and cholangiogenic sepsis
rarely there is time to disseminate
prognosis is generally poor
Carcinoma of extrahepatic bile ducts
- presents as progressive jaundice,
-less common than carcinoma of the gallbladder,
-men are more commonly affected
morphology: early produce extrahepatic obstructive jaundice- thus they
are only small in size, rarely metastasize- no time to develop meta
almost all are adenocarcinomas, more or less well-differentiated, usually
mucus secretion
clinically: all symptoms relate to bile duct obstruction-jaundice, weight
loss, pale stools, most carcinomas cannot be treated surgically at the time
of diagnosis
prognosis is poor- 5-year survival only 15%
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PATHOLOGY OF EXOCRINE PANCREAS
disorders of exocrine pancreas are relatively uncommon
there are three frequent lesions- acute pancreatitis, chronic
pancreatitis, and carcinoma
 acute pancreatitis
is characterized by acute onset of severe abdominal pain due to
enzymatic necrosis and inflammation of the pancreas
-it has a form of life-threatening acute hemorrhagic pancreatitis- fat
necrosis in and near the pancreas (Balsers necrosis)
morphology: -proteolytic destruction of pancreatic substance -necrosis of
blood vessels with subsequent hemorrhage, necrosis of fat by lipolytic
enzymes associated inflammatory reaction
grossly: blue-black hemorrhage interspersed throughut the pancreas and
foci of yellow -white fat necrosis
etiology: is still difficult to explain
-multiple predisposing factors have been described, such as
-association of gallstones and heavy chronic alcoholism
-possible role of reflux of bile to pancreas and activation of
pancreatic enzymes- obstruction of extrahepatic bile ducts
-role of obstruciotn of small intrapancratic ducts due to various
reasons
clinical course: main manifestation is severe abdominal pain -elevated
serum levels of enzymes, such as amylase, lipase
mortality rate is high even if the patient is operated
death is due to shock, secondary abdominal sepsis, adult respiratory
distress syndrome (shock lung)
 chronic pancreatitis - is characterized by repeated attacks of
inflammation
middle-aged men, mostly alcoholics are affected
morphology: fibrosing atrophy of exocrine gland tissue, with sparing of
Langerhans islets, often foci of dystrophic calcification, pseudocyststhis pattern is often associated with alcoholism
chronic obstructive pancreatiits- diffuse replacement of glands by
fibrous tissue- in main ecretory duct obstruction
clinical course: repeated attack of mild or moderate abdominal pain,
dyspepsia, chronic malabsorption
 carcinoma of pancreas
relatively frequent, most common in 6 to 8th decades
morphology: majority arise in the head of the pancreas, almost all lesions
are adenocarcinomas, some secret mucin, most tumors are desmoplastichard consistency
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carcinoma of the head presents early due to symptoms from common bile
duct obstruction (jaundice), in contrast - cancers of the body and the tail
of the pancreas are silent for longer time (except of pain)- usually
disseminate before diagnosis is establishedprognosis extremely poor- 5-year survival is not more than 2 %
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