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159 Cards in this Set

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PANCREAS, GALLBLADDER, AND BILIARY TRACT - LEE - FRIDAY FEB 16
ACUTE PANCREATITIS
what is acute pancreatitis, and what can it range from in severity?
a group of reversible lesions characterized by inflammation of the pancreas, ranging in severity from edema and fat necrosis to parenchymal necrosis with severe hemorrhage
how commin is acute pancreatitis?
relatively common
what is the most common cause, and what % of cases are due to this?
biliary tract disease and alcoholism (80%)
what % are idiopathic?
10-20%
what infections can cause acute pancreatitis (3)?
1) mumps; 2) coxsackieviruses; 3) Mycoplasma pneumoniae
what metabolic disorders can cause acute pancreatitis (2)?
1) hypertriglyceridemia; 2) hyperparathyroidism
inherited alterations in what types of genes can cause acute pancreatitis (2)?
1) pancreatic enzymes (cationic trypsinogen, PRSS1); 2) pancreatic enzyme inhibitors (trypsin inhibitor, SPINK1)
what can cause obstruction leading to acute pancreatitis (4)?
1) tumors; 2) pancreatic divisum (developmental anomaly); 3) parasites; 4) biliary sludge
what else can cause acute pancreatitis (3)?
1) trauma (surgery, blunt); 2) acute ischemia (thrombosis, embolism, vasculitis, shock); 3) medications
what is the pathogenesis of acute pancreatitis?
autodigestion of the pancreatic substance by inappropriately activated pancreatic enzymes
what is the last step before releasing activated enzymes into the pancreas?
acinar cell injury
what types of necrosis occur in acute pancreatitis?
1) fat necrosis; 2) parenchymal necrosis; 3) necrosis of blood vessels (with subsequent hemorrhage)
what else is seen in the pathology (2)?
1) edema; 2) acute inflammatory reaction
what are seen in severe cases (3)?
1) acute necrotizing pancreatitis; 2) hemorrhagic pancreatitis; 3) extensive extrapancreatic fat necrosis (omentum, mesentery)
what is the most predominant clinical feature?
abdominal pain
what type of pain is it, and where is it often felt?
constant and intense - often referred to the upper back
what is another clinical feature of acute pancreatitis?
peripheral vascular collapse and shock
what are three laboratory findings that may be abnormal?
1) serum amylase; 2) serum lipase; 3) hypocalcemia
what is the time progression of serum amylase changes (onset, peak, and return to normal)?
elevate 2-12 hours following onset, peak at 24-30 hours, return to normal in 3-4 days
what test has greater specificity than serum amylase, but is not frequently performed?
P-type (pancreatic) isoenzymes
what is time course and specificity of elevated serum lipase like, compared to serum amylase?
more specific, and time course is similar to amylase, but remains elevated for longer
what may hypocalcemia result from, and what significance does it have?
precipitation of calcium soaps in the fat necrosis - if it is persistent, it is a poor prognostic sign
how is acute pancreatitis managed?
"resting" the pancreas by total restriction of food and fluids, and by supportive treatment
what % of patients die early in the illness, and why?
5%, from shock
what other systemic problems/organ failures can occur (4)?
1) shock; 2) ARDS; 3) acute renal failure; 4) DIC
what other complications can occur (3)?
1) pancreatic abscess; 2) pseudocyst; 3) duodenal obstruction
PSEUDOCYSTS
what are pseudocysts?
localized collections of non-hemorrhagic material rich in pancreatic enzymes
what is lacking from pseudocysts?
epithelial lining
what processes are present in variable amounts (2)?
1) inflammation; 2) fibrosis
what are pseudocysts associated with (3)?
1) acute pancreatitis; 2) chronic pancreatisis; 3) trauma
what are complications of pseudocysts (4)?
1) abscess; 2) perforation; 3) peritonitis; 4) mass
CHRONIC PANCREATITIS
what is chronic pancreatitis?
irreversible destruction of exocrine pancreatic parenchyma by inflammation and fibrosis
what is destroyed in the late stages?
endocrine parenchyma
what is the most common cause?
long term alcohol abuse
what % are idiopathic?
40%
what are other causes (3)?
1) long term duct obstruction; 2) hereditary (PRSS1 or SPINK1 germline mutation); 3) tropical (possibly related to malnutrition)
what abnormality is idiopathic chronic pancreatitis associated with?
CFTR gene mutation (but no other clinical features of cystic fibrosis and normal sweat chloride test)
what are five main pathologic features of chronic pancreatitis?
1) atrophy of acini; 2) fibrosis; 3) chronic inflammation; 4) calcification/pancreatic calculi; 5) pseudocyst formation
CARCINOMA OF THE PANCREAS
what type of cancer is this, and where does it originate?
adenocarcinoma of the exocrine pancreas
how does it rank as far as leading causes of cancer death in the US?
4th
what is prognosis and 5 year survival rate?
extremely poor prognosis (less than 5% 5 year survival rate)
who does it occur in (race and age)?
elderly (80% of cases between ages of 60 and 80) and more common in blacks
what is the most important risk factor?
smoking
what diet increases risk?
high fat
what diseases increase risk?
1) chronic pancreatitis; 2) diabetes mellitus
what other substance increases risk?
alcohol
what familial syndromes increase risk (list)?
hereditary pancreatitis, Peutz-Jeghers syndrome, hereditary breast and ovarian cancer, HNPCC (Lynch II variant)
what is the most frequently altered oncogene in pancreatic cancer?
K-RAS
what is the most frequently inactivated tumor suppressor gene in pancreatic cancer?
P16 gene
what is the most common location in the pancreas for pancreatic carcinoma, and what % of cases arise there?
head: 60% (15% body, 5% tail, 20% diffuse)
what does pancreatic carcinoma look like grossly?
hard, gray-white, stellate, poorly defined masses
what does it look like microscopically, and what feature is seen?
adenocarcinoma, with dense stromal fibrosis ("desmoplastic reaction")
what are typical cinical features (list)?
weight loss, abdominal pain, back pain, anorexia, N/V, malaise, weakness
what other signs were mentioned as clinical features (2)?
1) progressive jaundice (90%); 2) Trousseau sign
what is Trousseau sign, and what % of cases does it occur in?
migratory thrombophlebitis - occurs in 10% of cases
what is it caused by?
elaboration of platelet-aggregating factors and procoagulants from tumor or its necrotic products
what is a serum marker for diagnosis and follow up?
CA 19-9
what other tools are used for diagnosis (2)?
1) CT or ultrasound-guided needle aspirate; 2) ERCP with brushings or biopsy
what % are resectable at the time of diagnosis?
<20%
what operation is performed, and what else is it known as?
pancreatoduodenectomy ("Whipple operation")
OTHER NEOPLASMS OF THE PANCREAS
what % of pancreatic neoplasms are cystic neoplasms?
5%
what types of cystic neoplasms must we know (2)?
1) serious cystadenoma; 2) mucinous cystic neoplasm
what other cystic tumors can occur in the pancreas (2)?
1) intraductal papillary mucinous neoplasm; 2) solid pseudopapillary tumor (solid-cystic tumor)
what is a serous cystadenoma, and what surrounds the cysts?
benign cystic neoplasm composed of glycogen-rich cuboidal cells surrounding small cysts
what are other names for a mucinous cystic neoplasm (2)?
1) mucinous cystadenoma; 2) cystadenocarcinoma
who do mucnous cystic neoplasms almost always arise in?
women
where in the pancreas do they arise?
tail
how malignant are they?
low grade (benign, borderline malignant)
what are they usually associated with?
dense stroma (similar to ovarian stroma)
what is the treatment?
distal pancreatectomy with splenectomy
what other neoplasm of the tumor occurs, and with what frequency?
pancreatic endocrine neoplasms (islet cell tumors): about 2%
CHOLELITHIASIS (GALLSTONES)
what % of adult populations in developed countries have gallstones?
10-20%
what does prevalence increase with?
age
what are the two main types of gall stones, and how prevalent (%) is each type?
1) cholesterol stone (80%); 2) pigment stone (primarily bilirubin - 20%)
what races are most likely to get cholesterol stones (3)?
1) Native American; 2) Mexican; 3) Northern European
what are the three biggest risk factors for cholesterol stones?
1) age; 2) female sex hormones (female gender, oral contraceptives, pregnancy); 3) obesity
what are associated conditions (4)?
1) rapid weight reduction; 2) gallbladder stasis; 3) inborn disorders of bile acid metabolism; 4) hyperlipidemia syndromes
what race is most likely to get pigment stones?
Asian
what may cause pigment stones (2)?
1) biliary nfections; 2) chronic hemolytic syndromes
what biliary infections may be responsible (2)?
1) liver fluke; 2) E. coli
what are the four contributing factors to cholesterol stone pathogenesis?
1) cholesterol supersaturation; 2) gallbladder hypomotility; 3) crystal nucleation; 4) acccretion
what types of cholesterol stones are there (2)?
1) pure; 2) mixed
what types of pigment stones are there (2)?
1) black; 2) brown
which type(s) of stone usually is solitary (one stone)?
pure cholesterol stone
which type(s) of stone is the largest?
pure cholesterol stone (often 2-4 cm)
which type(s) of stones are the smallest?
black and brown pigment stones (both 0.2-0.5 cm), mixed cholesterol stones are 0.2-3cm
what color are pure and mixed cholesterol stones?
pure are white-yellow, mixed are variable
what type(s) of stones are irregularly shaped?
black and brown pigment stones
what shape are pure cholesterol stones?
round-oval
what shape are mixed cholesterol stones?
faceted-round
what are the cut surfaces of pure cholesterol stones and mixed cholesterol stones like?
crystalline (both)
what are the cut surfaces ofblack pigment stones and brown pigment stones like?
black: crystalline/amorphous; brown: laminated
what % of cholelhitiasis cases are asymptomatic/silent?
80%
what type of pain do gall stones cause?
colicky pain - excruciating and constant
what is the risk of producing complications requiring surgery (within five years)?
30%
what are common complications of gall stones (must know - 4)?
1) cholecystitis (acute and chronic); 2) choledocholithiasis; 3) ascending cholangitis; 4) acute pancreatitis
what are uncommon complications (4)?
1) gallstone ileus; 2) biliary enteric fistula; 3) "porcelain gallbladder"; 4) hydrops of gallbladder
what is the most common treatment for gall stones?
laprascopic cholecystectomy (also can have open cholecystectomy, oral bile salt therapy, and percutaneous radiologic extraction)
ACUTE CHOLECYSTITIS
what types of acute cholecystitis are there (2)?
1) calculous; 2) acalculous
what causes acute calculous cholecystitis?
obstruction of neck or cystic duct
what % of cholecystitis are acalculous?
<5%
what can cause acute acalculous cholecystitis (list)?
postoperative state after major, nonbiliary surgery, trauma, burns, multisystem organ failure, sepsis, prolonged intravenous hyperalimentation, postpartum state, vasculitis
what is gallbladder like, and what is seen on the surface?
enlarged, edematous gallbladder, with fibrinous exudate on the surface
what other pathological features are seen (3)?
1) gangrenous cholecystitis; 2) empyema; 3) porcelain gallbladder
what is porcelain gallbladder?
deposition of calcium within the gallbladder wall
CHRONIC CHOLECYSTITIS
what may chronic cholecystitis be a sequel to?
repeated bouts of mini-acute cholecystitis
what % of the cases have gall stones?
more than 90%
what is the pathologic course like?
extremely variable, sometimes miniimal
what two features will be seen?
1) fibrosis; 2) chronic inflammation (variable degree)
what structure is characteristic of chronic cholecystitis (name and what they are)?
Rokitansky-Aschoff sinuses (mucosal diverticuli)
CHOLESTROLOSIS
what is another name for cholestrolosis?
"strawberry gallbladder"
what happens in cholestrolosis, and where?
focal accumulation of triglyceride and cholesterol-laden macrophages within the tips of mucosal folds
what is the clinical significance?
none
CARCINOMA OF THE GALLBLADDER
what sex and age does carcinoma of the gallbladder most commonly occur in?
slightly more common in women, in the 7th decade of life
what is the 5 year survival rate?
1% (rarely discovered at a resectable stage)
what % of cases have gallstones?
60-90%
what type of carcinoma is seen, and what feature does it have?
adenocarcinoma, with marked desmoplastic reaction
what % of cases have preoperative diagnosis of carcinoma?
<20%
CARCINOMA OF THE EXTRAHEPATIC BILE DUCTS
what is the presenting feature of carcinoma of the extrahepatic bile ducts?
painless, progressive jaundice
what age and sex is it most common in?
old men
what % of cases have gallstones?
33%
what diseases increase the risk of carcinoma of the extrahepatic bile ducts (3)?
1) primary sclerosing cholangitis; 2) ulcerative colitis; 3) choledochal cyst
what increases the risk in Asia?
biliary tree fluke infection (Clonorchis sinensis)
what type of carcinoma is seen, and what feature does it have?
adenocarcinoma, with marked desmoplastic reaction
what is the mean survival range for this disease?
6 to 18 months (usually not resectable