Ch 19

Question 1

what is a annular pancreas

Answer 1

congenital anomaly

abnormal rotation of the pancreas

The central pancreas may have two lobes that migrate in the opposite direction to fuse w/ the dorsal portion

can lead to duodenal obstruction

Question 2

what is a pancreas divisum

Answer 2

MC congenital anomaly due to the failure of ventral and dorsal bud fusion after rotation

The ventral/dorsal pancreas do not join/fuse w/ the ductal system, –> not a shared ductal system w/ the pancreas and CBD

associated w chronic pancreatitis

Question 3

what are the pathogenesis pathways for acute pancreatitis

Answer 3

1. toxic release of enzymes, cytokines and other mediators into the circulation–> cause SIR - increase WBC, DIC, edema, and ARDS. Shock may occur bc the SIR syndrome and acute renal tubular necrosis
2. Once tissue damage begins, trypsin can directly/indirectly active factors in the blood (coagulation, complement, kallikrein and fibrinolytic pathways) –> inflam and small-vessel thrombosis –> further damage to acinar cells and amplify intrapancreatic enzyme activation

Question 4

what do acute and chronic pancreatitis have in common

Answer 4

due to injury due to autodigestion by its own enzymes when protective mechanisms are disrupted/overloaded

Question 5

what is the etiology and prognosis of acute pancreatitis

Answer 5

western countries

w/ alcoholism - > men

w/ biliary tract dz- > women

The systemic organ failure and pancreatic necrosis are prognostic indicators

Question 6

what is the presentation of acute pancreatitis

Answer 6

Abd pain is the cardinal manifestation

= constant, intense, and may refer to the upper or mid back and occasionally to the L shoulder

The pt may also present w/ cullen’s sign (through round L) and grey turner’s sign (subQ tissue)

Question 7

what are cullen and grey turner signs

how do they occur

what info do they provide

Answer 7

cullen’s sign (through round L) periumbilical ecchymosis

grey turner’s sign (subQ tissue) flank ecchymosis

increased pancreatic enzymes –> diffuse necrosis and inflam w/ retroperitoneal and intraabd bleeding.

The cullens and grey turner signs are not specific, however they are associated w/ severe acute pancreatitis and high mortality

Question 8

what is relationship of gallstones and acute pancreatitis

Answer 8

Gallstones are present in 35-60% acute pancreatitis cases and can cause gallstone pancreatitis in about 5% of pts

Question 9

what labs/imaging studies are used to assess acute pancreatitis

Answer 9

elevated lipase (most specific and sensitive marker) and also elevated amylase (which has a short t1/2 so may return to normal before lipase doesn).

10% of cases have glycosuria, decreased Ca2+ due to saponification of necrotic fat

CT scan for direct visualization of the enlarged/inflamed pancreas (at levels L1-L4)

Question 10

what will be seen on imaging for severe acute cases of pancreatitis

what is the outome

Answer 10

CT will present w/ necrotizing pancreatitis w/ several acute peripancreatic fluid collections AND gallstones in GB w/ NO choledocholithasis

• multiple organ system failure and the pt may pass from complications of pancreatitis even w/ supportive care

Question 11

what is the morphology of acute pancreatitis

Answer 11

ranges from limited inflam/edema to extensive necrosis and hemorrhage

the extent of injury is based on the duration/severity of the dz

The basic changes in morphology include: microvascular leak/edema, fat necrosis, acute inflam, damage/ autodigestion of pancreatic parenchyma and BV destruction w/ intestinal hemorrhage

Question 12

where does fat necrosis occur in acute pancreatitis

Answer 12

in the omentum and mesentery of the bowel next to the pancreas and beyond the abd cavity (subQ fat) bc of systemic lipase release

The peritoneal cavity contains serous, slightly turbid, brown-tinged fluid w/ fat globules that reflect digestion of adipose tissue

Question 13

what is the Tx for acute pancreatitis

Answer 13

put the pancreas to “rest.”

NPO, supportive IV fluids and analgesia

Most pt recover however 5% w/ severe cases die w/i the first week of illness.

Question 14

what are complications of continued damage in acute pancreatitis

Answer 14

sterile pancreatic abscesses and pancreatic pseudocysts

In 40-60% pt w/ acute necrotizing pancreatitis, the acellular debris can become infected by gram (-) organism

ARDS and acute renal failure

.

Question 15

what is the cause of chronic pancreatitis

Answer 15

prolonged inflam of the pancreas associated w/ irreversible destruction of the exocrine parenchyma, fibrosis and in late stages loss of endocrine parenchyma

The MCC is long term EtOH use

TGF-B is produced by activated macrophages

Question 16

what is the fxn of TGF-B in chronic pancreatitis

Answer 16

scar formation and fibrosis of the lung, liver and kidneys

also = anti-inflam cytokine so it limits and stops the inflam response by inhibiting lymphocyte prolif and the activity of other leukocytes

Question 17

what increases suspicion of chronic pancreatitis?

what is the prognosis

Answer 17

are repeated occurrences of abd pain, persistent back pain, calcifications, pseudocyst, pancreatic exocrine insuf and DM

poor long-term outcome, with 50% mortality rate 20-25 yrs later

The significant morbidity/mortality can be due to the pancreatic exocrine insuf, chronic malabs and DM

Question 18

what is AI pancreatitis

Answer 18

Type 1 =IgG4 which secrete plasma cells in the pancreas and is one of the manifestations of IgG related dz

Type 2 is restricted to the pancreas, except in a few forms of UC

Both types may mimic pancreatic carcinoma and present as a mass lesion in the pancreatic head on imaging

Question 19

how does inheritance come into play for pancreatitis

what genes may be involed and what are their fxns

Answer 19

hereditary factor = defect that causes increase/sustains the activity of trypsin

present w/ recurrent attacks of severe acute pancreatitis beginning in childhood

Pt w/ PRSS1 mutations have a 40% increased risk for developing pancreatic CA.

Question 20

what are congenital cyst

Answer 20

thin-walled cysts that result from anomalous pancreatic duct development

A thining fibrous capsule filled w/ clear serous fluid

may be sporadic/inherited conditions, such as AD polycystic kidney dz and von hippel-lindau dz

Question 21

what are pseudocysts

Answer 21

= most of the pancreatic cysts

The lack an epithelial lining and form after multiples recurrences of acute pancreatitis, esp when superimposed on chronic alc pancreatitis

can also be due to trauma

resolve spontaneously, become infected or compress/perforate neighboring structures

Question 22

what are the neoplasms of the pancreas that appear cystic

Answer 22

serous cystic neoplasm

mucinous cystic neoplasm

solid-pseudopapillary neoplasm

intraductal papillary mucinous neoplasms

Question 23

what are characteristics of Serous cystic neoplasms

Answer 23

benign, F, 60-70s, pancreatic tail

Question 24

what are characteristics of Mucinous cystic neoplasms:

Answer 24

benign, borderline or carcinoma, 95% F, pancreatic tail

Question 25

what are characteristics of Solid-pseudopapillary neoplasm:

Answer 25

young F, pancreatic tail, locally aggressive

Question 26

what are characterisitics of Intraductal papillary mucinous neoplasms: (IPMNs):

Answer 26

connected to duct system, MALE, pancreatic HEAD and progress to CA

Question 27

explain the progression of PanIN to invasive carcinoma

Answer 27

(Invasive CA arise form noninvasive precursor lesions (pancreatic intraepithelial neoplasia -PanIN))

Question 28

what is the etiology of pancreatic carcinoma

Answer 28

> 60 yo,

african american, japanese american, native american, hawaiian and ashkenazi jew

Question 29

what is the prognosis of pancreatic carcinoma

Answer 29

Survival after dx of advanced pancreatic CA is short

> 80% cases are unresectable by the time of dx bc of the invasion of vessels and other structures/distant metastasis

However pt who have a tumor that can be successfully resected can survive a longer period

Question 30

what is the use of CA 19-9 and carcinoembryonic antigen

Answer 30

the longer survival w/ resection of the tumor shows the importance of early detection

However there is no marker for pancreatic CA for detection

CA 19-9 and carcinoembryonic antigen: lacks specificity and sensitivity- It is seen to be elevated in pt w/ pancreatic CA and is useful in monitoring response to tx.

Question 31

what are the RF for pancreatic carcinoma

Answer 31

cigarette smoke (doubles risk of pancreatic CA)

chronic pancreatitis (association of chronic inflam, tissue repair and neoplasia)

visceral obesity and high body mass index

DM (modest RF)

Question 32

how do pancreatic carcinomas present

Answer 32

silent until invasion to neighboring structures

Pain is usually the first sign but by the time the pain appears the CA is far beyond curabl

wt loss, anorexia and generalized malaise and weakness tends to be a sign for advanced dz.

migratory thrombophlebitis (trousseau sign)

Tumor associated inflam and coagulation factors from tumor cells cause increased risk of thromboembolism and disseminated CA

Question 33

what is the morphology of pancreatic carcinoma

Answer 33

adenocarcinoma and a dense desmoplastic reaction

Question 34

where do the most of the tumors occur on the pancreas

how does this affect presentation

Answer 34

60% of cancers present in the head of the gland, which are associated w/ obstructive jaundice

These obstruct the distal CBD –> distention of the biliary tree = Courvoisier sign- palpable GB w/ mild painless jaundice

Question 35

besides the head of the pancreas, where can tumors occur on the pancreas

Answer 35

15% present in the body and 5% in the tail. 20% may involve the whole gland.

Question 36

what are common sites of invasion for pancreatic carcinoma

Answer 36

tends to grow along Ns and invade BVs and retroperitoneum

The perineural, lymphatic and large vessel invasion is common

direct invasion- spleen, adrenals, transverse colon and stomach

peripancreatic, gastric, mesenteric, omental and portohepatic LNs are commonly involved as well

Distant metastasis = liver and lungs