Pancreas II: Cystic Fibrosis, Cystic Disorders, and Pancreatic Cancer

Question 1

3 main symptoms of CF

Answer 1

**Lung Infections/obstruction/infalmmation
**Chronic Pancreatitis
**Hypertonic Sweat
Heptobilliary tract dz
Meconium ileus in infants
infertility

Question 2

genetic mutation causeing CF

Answer 2

three base pair deletion (∆DF508) that results in defective folding and processing of CFTR Cl- channel

Question 3

CFTR is a (IP3 or cAMP) activated Cl- channel

Answer 3

cAMP

Question 4

what is the pathophys of CF

Answer 4

defective CFTR Cl channel → defefctive Cl- secretion → thickening of secretions → obstruction of ducts and lumens → infection, inflammation and tissue destruction

Question 5

acini in CF are

Answer 5

atrophic

Question 6

gross changes to pancreas in CF

Answer 6

replacement of exocine portion of pancreas with FAT

Question 7

What clinical symptoms diagnose CF

Answer 7

frequent pulm infections (pseudomonas)
Diarrhea, malasb (steatorrhea), and failure to thrive
Dehydration (freq episones)

Question 8

testing used to diagnose CF

Answer 8

sweat Cl- test
PFTs
Genetic Testing

Question 9

How is CF managed

Answer 9

Daily Pulm Toilet/Inhalers that loosen secretions
high dose pancreatic enzymes
treat pulm infections

Question 10

what is the pathophys of the failure to thrive seen in CF?

Answer 10

no acinar cells = no pancreatic digestive enzymes

Question 11

most common cyst-like lesion in the pancrease

Answer 11

pseudocyst

Question 12

In general, of the neoplastic cysts __ _have very little malignant potential while ___ have malignant potential

Answer 12

serous = not malignant
mucinous = malignant

Question 13

what are 4 examples of cystic neoplasms of the pancreas. which are benign and malignant?

Answer 13

Benign:
Serious cystic tumors

Malignant:
Mucinous Cyst neoplasms
Intraductal Papillary mucinous neoplasms
solid pseudopapillary Neoplasms

Question 14

CEA is a tumor marker for

Answer 14

mucinous cystic neoplasm

Question 15

ERCP is a tumor marker for

Answer 15

intraductal papillary mucinous neoplasms

Question 16

common presentation of pancreatic cancer

Answer 16

weight loss, abdominal pain, jaundice

Question 17

90% of pancreatic cancers are

Answer 17

ductal adenocarcinomas

Question 18

What are the miscellaneous pancreatic cancers that account for the other 10%

Answer 18

cystic neoplasms
acinar cell neoplasms
lymphomas and sarcomas
neuroendocrine tumors (gastrinoma, insulinomas, glucagonomas)

Question 19

not going into the histology

Answer 19

nichols made it sound like even pathologists struggle with it

Question 20

Risk factors for pancreatic cancer

Answer 20

smoking
alcohol in the setting on chronic pancreatitis
hereditary pancreatitis
family history of pancreatic cancer

Question 21

What is the tumor marker for pancreatic adenocarcinoma

Answer 21

CA19-9 **DUCTAL cell tumor marker

Question 22

pathophys of jaundice in pancreatic adenocarcinoma

Answer 22

obstruction of CBD

Question 23

What clinical presentation is a clue that the pt may have a mass in the head of their pancreas

Answer 23

painless jaundice

**60-70% of pancreatic adenocarcinomas are in the head of the pancreas

Question 24

what surgical options can cure some pancreatic cancers?

Answer 24

distal pancreatectomy (tail lesions)
Whipple resection (for head lesions)

**chemo and radiation have limited effectiveness therefore surgery is the only chance for a cure

Question 25

what is the best prognostic factor for pancreatic cancers

Answer 25

retroperitoneal resection margin

**only for resectable adenocarcinomas in the head of the pancreas