Pancreas

Question 1

is caused by a failure of fusion of the fetal duct systems of the dorsal and ventral pancreatic primordia.

Answer 1

Pancreas Divisum

Question 2

is a band-like ring of normal pancreatic tissue that completely encircles the second portion of the duodenum.

Answer 2

Annular Pancreas

Question 3

Pancreatic tissue that is aberrantly situated, found in about 2% of careful routine postmortem examinations

Answer 3

Ectopic pancreas
## FOOTNOTE

Favored sites: stomach, duodenum, jejunum, Meckel diverticula, ileum

Question 4

Occurs when the pancreas fails to develop caused by germline mutations

Answer 4

Agenesis

Question 5

A gene encoding a homeobox transcription factor that is critical for pancreatic development

Answer 5

PDX1

Question 6

characterized by reversible pancreatic parenchymal injury and inflammation and has many causes, including toxic exposures (e.g., alcohol), pancreatic duct obstruction (e.g., biliary calculi), inherited genetic defects, vascular injury, and infections.

Answer 6

Acute pancreatitis

Question 7

Alcoholic male to female ratio of acute pancreatitis

Answer 7

6:1

Question 8

Male to female ration of acute pancreatitis with biliary tract disease

Answer 8

1:3

Question 9

Most commonly cause of pancreatic duct obstruction

Answer 9

Gallstones and biliary sludge

Question 10

Pancreatic enzyme secreted in its active form which has the potential to cause local fat necrosis

Answer 10

Lipase

Question 11

leads to release of digestive enzymes, inflammation, and tissue autodigestion.

these result in oxidative stress and generation of intracellular free radicals that lead to membrane lipid oxidation and transcription factor activation.

Answer 11

Primary acinar cell injury

Question 12

important trigger for inappropriate enzyme activation

Answer 12

Calcium flux

Question 13

Two factors have been proposed to contribute to alcohol- indcuced pancreatitis. These are

Answer 13

Ducts obstruction
Acinal cell damage

Question 14

The feature shared by most forms of hereditary pancreatitis is a defect that increases or sustains the activity of trypsin . Three genes are implicated and these are

Answer 14

PRSS1, SPINK1 and CFTR

Question 15

Most hereditary cases are due to gain-of-function mutations in the trypsinogen gene known as

Answer 15

PRSS1

Question 16

This gene, when affected by loss-of-function mutations, can contribute to hereditary pancreatitis by failing to inhibit trypsin?

Answer 16

SPINK1

Question 17

cystic fibrosis is caused by mutations in the

Answer 17

Cystic fibrosis transmembrane conductance regulator (CFTR)

Question 18

the cardinal manifestation of acute pancreatitis

Answer 18

Abdominal pain

Question 19

the most specific and sensitive marker of acute pancreatitis

Answer 19

Serum lipase

Question 20

prolonged inflammation of the pancreas associated with irreversible destruction of exocrine parenchyma, fibrosis, and, in the late stages, loss of endocrine parenchyma.

Answer 20

Chronic pancreatitis

Question 21

Most affected patients of chronic pancreatitis

Answer 21

Middle aged males

Question 22

most common cause of chronic pancreatitis is

Answer 22

Long term alcohol use

Question 23

fibrogenic cytokines that induce activation and proliferation of pancreatic stellate cells (periacinar myofibroblasts), collagen deposition, and fibrosis

Answer 23

TGFB and PDGF

Question 24

Autoimmune pacreatitis that is associated with the presence of immunoglobulin G4 (IgG4)–secreting plasma cells in the pancreas and is one manifestation of a systemic IgG-related disease

Answer 24

Autoimmune pancreatitis type 1

Question 25

Autoimmune pancreatitis that is restricted to the pancreas with the exception of a subset of patients with ulcerative colitis.

Answer 25

Autoimmune pancreatitis type 2

Question 26

are **unilocular, thin-walled cysts** that likely result from anomalous pancreatic duct development.

Answer 26

Congenital cysts

Question 27

formed when areas of **intrapancreatic or peripancreatic** hemorrhagic fat necrosis are walled off by fibrosis and granulation tissue

Answer 27

Pseudocysts ## FOOTNOTE they lack an epithelial lining

Question 28

usually occur in the tail of the pancreas. The cysts are small (1 to 3 mm) and can be solitary, multiple, or present as a honeycomb of microcystic lesions. These cysts are lined by glycogen-rich cuboidal cells, and contain clear, thin, straw-colored fluid

Answer 28

Serous cystic neoplasms

Question 29

is the most common genetic abnormality in serous cystic neoplasms

Answer 29

Inactivation of VHL tumor suppressor gene

Question 30

arise in women and, in contrast to serous cystic neoplasms, they are precursors to invasive carcinomas.

Answer 30

Mucinous cystic neoplasms

Question 31

Mucinous cystic neoplasms arise in the

Answer 31

Tail of the pancreas ## FOOTNOTE Present as painless, slow-growing masses

Question 32

Cystic cavities are larger, filled with **thick, teancoius mucin**

Answer 32

Mucinous cystic neoplasms

Question 33

Epithelial lining of mucinous cystic neoplasm

Answer 33

Columnar

Question 34

Mutations involved in mucinous cystic neoplasm

Answer 34

KRAS (majority) TP53 SMAD4

Question 35

are mucin-producing neoplasms that involve the larger ducts of the pancreas

Answer 35

Intraductal papillary mucinous neoplasms

Question 36

arise more frequently in men and tend to involve the head of the pancreas

Answer 36

Intraductal papillary mucinous neoplasms

Question 37

2 useful features that distinguish IPMN from mucinous cystic neoplasms

Answer 37

Absence of ovarian stroma Involvement of a pancreatic duct

Question 38

the third leading cause of cancer deaths in the United States, also known as infiltrating ductal adenocarcinoma of the pancreas

Answer 38

Pancreatic carcinoma

Question 39

Invasive pancreatic cancers arise from noninvasive **precursor lesions** referred to as

Answer 39

Pancreatic intraepithelial neoplasia

Question 40

the most frequently altered oncogene in pancreatic cancer, with activating point mutations present in greater than 90% of cases.

Answer 40

KRAS (chromosome 12p)

Question 41

usually the first symptom of carcinomas of the pancreas

Answer 41

Pain

Question 42

It is associated with most cases involving the pancreatic head, as these tend to block the common bile duc

Answer 42

Obstructive jaundice

Question 43

A palpably enlarged, nontender gallbladder with mild painless jaundice

Answer 43

Courvoisier sign

Question 44

Migratory thrombophlebitis, known as the

Answer 44

Trousseau sign

Question 45

form zymogen granules and produce exocrine enzymes such as trypsin and lipase

Answer 45

Acinar cell carcinoma

Question 46

rare neoplasms that occur primarily in children 1 to 15 years of age. They have a distinct microscopic appearance consisting of squamous islands admixed with acinar cells

Answer 46

Pancreatoblastoma

Question 47

Mutations involved in the acinar cell carcinoma

Answer 47

APC CTNNB1