acute and chronic pancereatitis (pathology)

Question 1

pancreas parenchima

Answer 1

80% exocrine

1-2% langrhans islands, endcrine

Question 2

exocrine cells

Answer 2

acinic cells
proenzymes: trypsinogen + chymotrypsinogen
active enzemys: amylase+ lipase

ductular (small ducts) cells: bicarbonate 2-7mm

ductal (large ducts) cells: mucin

Question 3

exocrine self digestion defense mechanism

Answer 3

proenzymes
zymogenic granular secrition
entrokinase dependant activation

Question 4

chymotryapsinogen activator?

Answer 4

tyrapsin

Question 5

trypsinogen activator?

Answer 5

entrokinase in brush border

trypsin (high ca+2)

Question 6

trypsin inhibition?

Answer 6

acinar and ductal secretions: serine protease inhibitor kazal type 1 (SPINK1)

auto self inhibition of trypsin (low ca+2)

Question 7

acute pancreatitis etiology

Answer 7

metabolic: alchohol, drugs, hyperlipoproteinemia

mechanical:
obstructions- gallstones,tumors
injuries- endoscopic

vascular- shock, emboli

infections- mumps, m.pneumoniae

idiopathic

Question 8

genetic etiology of acute pancreatitis

Answer 8

cationic trypsinogen (serine protease 1 PRSS1, 7q35)
GOF- self inactivation cleavage site germline mutation
AD 0.8 penetrance

CFTR (7q31)
LOF
duct obstruction

SPINK1
GOF
AR

Question 9

etiology of acute pancreatitis

Answer 9

30-60% due to gall stones

alcholism

Question 10

acute pancreatitis - duct obstruction pathopysiology

Answer 10

obstruction
interstitial edema
impaired blood flow 
ischemia
acinar cell injury
activation of enzymes

Question 11

acute pancreatitis -defective intracellular transport pathopisiology (not prooven yet)

Answer 11

transport of pancreatic proenzymes to lysosome

activation of enzymes

Question 12

alchol (usually chronic) induced acute pancreatitis pathopysiology

Answer 12

contraction of spinchter of oddi-obstruction

higher exocrine viscosity- obstruction

free radicals -> ca+2 is up -> self activation of trypsin

Question 13

acute pancreatitis morphology

Answer 13

microvascular leakage -edema

necrosis of fat (lypase and amylase endocrinic dissemination) ->
saponification- ca+2 + phospholipds addhition

destruction of BV- hemmorage

Question 14

chronic pancreatitis 2 types

Answer 14

calcific / obstructive

autoimmune

Question 15

calcific / obstructive chronic pancreatitis definition

Answer 15

chronic inflammation with irreversible changes in structure which lead to 100% loss of exocrine activity

Question 16

calcific / obstructive chronic pancreatitis etiology

Answer 16

long standing obstruction
chronic alchoholism 10-20
smoking (strongest advansor of acute to chronic pancreatitis)

Question 17

calcific / obstructive chronic pancreatitis chimokins

Answer 17

IL 8
TGFb
PDGF
all proliferate myofibroblasts -> collagen deposition and fibrosis

Question 18

calcific / obstructive chronic pancreatitis morphology

Answer 18

fibrosis and chronic inflammation infiltrations
acini atrophy
relative sparing of endocrin pancease
dialition of ducts

Question 19

PRSS1 heredetary pancreatitis morphology

Answer 19

lipomatous atrophy

Question 20

autoimmune chronic pancreatitis types

Answer 20

type 1-
elevated IGg4
infiltration by IGg4 plasma cells (peri-ductal!)
obliterative phlebitis- narrowing venuls due to inflammation and fibrosis
no calcification in ducts

type 2-
neutrophilic epithelial infiltration (granulocytic epithelial lessions)

both exocrine and endocrine disfunction

Question 21

autoimmune chronic pancreatitis treatment

Answer 21

prominant recover after oral steroids

Question 22

pancreatic pseudocyst

Answer 22

necrotic hemorrhagic material rich in p.enzymes
solitary
no epithel - “pseudo”

Question 23

pancreatic pseudocyst locations

Answer 23

in pancease

peripancreatic tissue
lesser omental sac
retroperitoneal

Question 24

pancreatic neoplasms

Answer 24

endocrine

exocrine
solid
cyctic

Question 25

pancreatic cyctic neoplasms

Answer 25

solid pseudopapillary tumor serous cyst neoplasm mucinous cytic neoplasms intraductal papillary mucinuos neoplasms (IPMN)

Question 26

solid pseudopapillary tumor epidimeology

Answer 26

young females | childhood

Question 27

solid pseudopapillary tumor morpholgy

Answer 27

large (abdominal discomffort) resective mostly bening

Question 28

solid pseudopapillary tumor etiology

Answer 28

CTNNB1

Question 29

serous cyst neoplasm epidemiology

Answer 29

M:F 2:1 | 60's

Question 30

serous cyst neoplasm favored lacation

Answer 30

2/3 in body tail region

Question 31

serous cyst neoplasm morphology

Answer 31

``` many tightly packed small thin walled cyts (da!) honecomb appearance ```

Question 32

serous cyst neoplasm etiology

Answer 32

von hipple-lindau gene mutes 40% of cases

Question 33

IPMN epidemiology

Answer 33

M3:F2 | 60's

Question 34

IPMN morphology

Answer 34

main pancreatic duct of branches , mostly in head dialition of ducts (grossl visible) papillary intraductal mucin producing epithelial tumors (adenoma to cacinoma insitu)

Question 35

IPMN etiology

Answer 35

Petz-heghers familial adenomatous polyposis syndromes 1/3 with history of malignancy (aspecialy gastric or colonic) GNAS, 80%, 20q13, alfa stimulatory G protein subunit GOF KRAS @ RNF43 TP53 @ SMAD4 when invasive

Question 36

mucinous cytic neoplasms epidemiology

Answer 36

females | perimenopausal

Question 37

mucinous cytic neoplasms favord location

Answer 37

tail | body

Question 38

mucinous cytic neoplasms etiology

Answer 38

RNF43 KRAS TP53

Question 39

pancreatic ductal carcinoma epidemiology

Answer 39

``` mostly elderly (60's >80%) 5yr survival <5% familial clusters (10% of cases) ```

Question 40

pancreatic ductal carcinoma causes (risks)

Answer 40

smoking chronic pancreatitis heredetary pancreatitis (40% life time risk) obesity and diabetes

Question 41

pancreatic ductal carcinoma familial clusters etiology

Answer 41

``` HNPCC (lynch syndrome) BRCA2, PALB2 (breast and ovary cancer) Peutz-jegher li fraumeni (p53) CDKN2A ```

Question 42

pancreatic ductal carcinogenesis precursor lessions

Answer 42

IPMN Mucinous cystic neoplasms pancreatic intraepithelial neoplasias (PanIN)-90%!

Question 43

pancreatic ductal carcinoma mulecular alternations

Answer 43

kras 90% oncogene cdkn2a 30% smad4 55% supressor p53 60% supressor