19-1: Pancreas Flashcards

1
Q

what are the primary roles of the pancreas?

A

exocrine – secretes enzymes for digestion (trypsinogen, chymotrypsinogen, procarboxypeptidase, etc.)

endocrine – secretes insulin, glucagon, and somatostatin

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2
Q

what is the most common congenital anomaly of the pancreas?

A

pancreas divisum due to failure of ventral and dorsal bud fusion

*associated with chronic pancreatitis

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3
Q

what complication is associated with a congenital annular pancreas?

A

duodenal obstruction

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4
Q

what is an ectopic pancreas?

A

a congenital anamoly in which the pancreas is formed within an abnormal place

usually stomach or duodenum

can cause pain or mucosal bleeding

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5
Q

what are some pancreatic protective mechanisms that prevent autodigestion?

A

enzymes are synthesized as inactive proenzymes and packaged within secretory granules

proenzymes are not activated until they come into contact with activated trypsin. Trypsin is activated by enterokinase within the duodenum

acinar and ductal cells secrete trypsin inhibitors

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6
Q

what is the clinical presentation of acute pancreatitis?

A

constant, intense, upper or bid back pain
occasionally radiates to left shoulder
anorexia
N/V

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7
Q

what lab findings are associated with acute pancreatitis?

A

elevated serum amylase and lipase

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8
Q

what lab finding is most specific for acute pancreatitis?

A

lipase - remains elevated for 8-14 days

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9
Q

what is the etiology of acute pancreatitis?

A

release of toxic enymes and cytokines activating a systemic inflammatory response

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10
Q

what PE findings are highly associated with high mortality in severe acute pancreatitis?

A

Cullen’s sign (periumbilical ecchymosis)

Grey Turner’s sign (flank ecchymosis)

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11
Q

what metabolic processes are associated with pancreatitis?

A

alcohol
hyperlipoproteinemia
hypercalcemia
drugs

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12
Q

what genetic mutations are associated with pancreatitis?

A

PRSS1 - encodes trypsin
SPINK1 - encodes trypsin regulators
CASR - encodes calcium metabolism regulators

CFTR

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13
Q

what mechanical processes are associated with pancreatitis?

A
gallstones
trauma
iatrogenic injury
operative injury
endoscopic procedures with dye injection (ERCP)
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14
Q

what vascular processes are associated with pancreatitis?

A

shock
atheroembolism
vasculitis

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15
Q

what infectious agents are associated with pancreatitis?

A

Mumps

Coxsackievirus (of course)

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16
Q

what is the pathogenesis of pancreatitis?

A

after tissue damage occurs, trypsin can directly or indirectly activate factors found in the blood

the resulting inflammation and small-vessel thrombosis causes further damage to the acinar cells which amplifies intrapancreatic enzyme activation

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17
Q

what are the basic abnormalities associated with pancreatitis?

A
microvascular leak and edema
fat necrosis
acute inflammation
autodigestion of pancreatic parenchyma
blood vessel destruction and interstitial hemorrhage
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18
Q

what are some possible systemic complications of pancreatitis?

A

acute respiratory distress syndrome

acute renal failure

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19
Q

what is the definition of chronic pancreatitis?

A

prolonged inflammation of the pancreas associated with irriversible destruction of exocrine parenchyma, fibrosis and eventual loss of endocrine parenchyma

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20
Q

what is the most common cause of chronic pancreatitis?

A

long-term alcohol use (yikes, we’re in trouble)

21
Q

what cytokine is associated with chronic pancreatitis?

A

TGF-B – involved in scar formation and fibrosis

22
Q

what is autoimmune pancreatitis type 1?

A

associated with the presence of IgG4 secreting plasma cells in the pancreas

one manifestation of systemic IgG related disease

23
Q

What is autoimmune pancreatitis type 2?

A

restricted to the pancreas except in a subset of patients with ulcerative colitis

24
Q

what is the clinical presentation of autoimmune pancreatitis?

A

mimic pancreatic carcinoma

mass lesion in the pancreatic head on imaging

25
Q

what is the clinical presentation of chronic pancreatitis?

A

repeated bouts of abdominal pain
persistent back pain

on imaging: calcifications and pseudocysts

labs: pancreatic exocrine insufficiency and DM

26
Q

what mainfestations of chronic pancreatitis lead to morbidity and contribute to mortality?

A

pancreatic exocrine insufficiency
chronic malabsorption
DM

27
Q

patients with herediatry pancreatitis associated with PRSS1 have a risk of developing what?

A

pancreatic cancer

28
Q

what are congenital pancreatic cysts?

A

thin-walled cysts that likely result from anamalous pancreatic duct development

extremely rare

29
Q

what are pancreatic pseudocysts?

A

arise following a bout of pancreatitis - usually from chronic alcoholic pancreatitis

lack an epithelial lining

many resolve spontaneously but some may become secondarily infected, compress adjacent structures or perforate

30
Q

what are serous cystic neoplasms?

A

always benign
F > M
60 y/o +
most common in the pancreatic tail

31
Q

what are mucinous cystic neoplasms?

A

95% women
may be precursors to invasive carcinoma
usually located in the pancreatic tail

32
Q

what are solid-pseudopapillary neoplasms?

A

young women
pancreatic tail
locally agressive malignancy

33
Q

what are intraductal papillary mucinous neoplasms (IPMNs)?

A

mucin-producing neoplasms that involve the duct of the pancreas
M > F
located in the pancreatic head
precursor to invasive carcinoma

34
Q

what demographics are associated with pancreatic cancer?

A

60 y/o +

African Americans, Japanese Americans, Native Americans, Hawaiian islanders and Ashkenazi Jews

35
Q

what risk factors are associated with pancreatic cancer?

A

cigarette smoking (2x risk)
chronic pancreatitis
visceral obesity and high BMI
DM

36
Q

what hereditary conditions have an increased risk of pancreatic cancer?

A
PJ syndrome (STK11)
Hereditary pancreatitis (PRSS1 or SPINK1)
Familial atypical multiple mole melanoma syndrome (CDKN2A)

Family hx of pancreatic cancer (> 3 relatives)
Hereditary breast and ovarian cancer
Hereditary non-polyposis colorectal cancer

37
Q

which gene mutations are highly associated with pancreatic ductal carcinoma?

A

KRAS
TP53
CDKN2A
SMAD4

38
Q

where are pancreatic cancers most likely to occur?

A

in the head of the pancreas

39
Q

why are pancreatic cancers hard to detect?

A

they are asymptomatic until they invade into adjacent structures

40
Q

what are the classic presentations of pancreatic cancer?

A

pain
weight loss
anorexia
general malaise and weakness

41
Q

what symptoms are associated with a tumor within the head of the pancreas?

A

obstructive jaundice caused by the head of the pancreas obstructing the distal common bile duct

Courvoisier sign - a palpably enlarged, nontender gallbladder with mild, painless, jaundice

42
Q

what is the growth pattern of pancreatic cancer?

A

grows along nerves and invades into blood vessels and the retroperitoneum

43
Q

what is frequently involved in pancreatic cancer via direct invasion?

A
spleen
adrenals 
transverse colon
stomach
local lymph nodes
44
Q

where are primary pancreatic metastases most common?

A

liver and lungs

45
Q

what are the microscopic features of pancreatic adenocarcinomas?

A

dense desmoplasic reaction

46
Q

what is Trousseau sign?

A

migratory thrombophlebitis - caused by elaboration of platelet activating factors and procoagulants from the tumors or its necrotic products

47
Q

what is the precursor lesion associated with pancreatic cancer?

A

pancreatic intraepithelial neoplasia (PanIN)

often found in the pancreatic parenchyma

microscopically show dramatic telomere shortening

48
Q

what is the model of progression from normal tissue to invasive carcinoma in the pancreas?

A
  1. normal pancreas
  2. PanIN-1A: telomere shortening
  3. PanIN-1B: KRAS activation
  4. PanIN-2: CDKN2A inactivation
  5. PanIN-3: TP53, SMAD4, and BRCA2 inactivation
  6. invasive carcinoma
49
Q

what is the prognosis of pancreatic cancer?

A

typically survival after diagnosis is unlikely (usually die within 1-2 years)

> 80% of pancreatic cancers are unresectable at the time of diagnosis due to invasion