Pancreas II: Cystic Fibrosis, Cystic Disorders, and Pancreatic Cancer Flashcards
3 main symptoms of CF
**Lung Infections/obstruction/infalmmation
**Chronic Pancreatitis
**Hypertonic Sweat
Heptobilliary tract dz
Meconium ileus in infants
infertility
genetic mutation causeing CF
three base pair deletion (∆DF508) that results in defective folding and processing of CFTR Cl- channel
CFTR is a (IP3 or cAMP) activated Cl- channel
cAMP
what is the pathophys of CF
defective CFTR Cl channel → defefctive Cl- secretion → thickening of secretions → obstruction of ducts and lumens → infection, inflammation and tissue destruction
acini in CF are
atrophic
gross changes to pancreas in CF
replacement of exocine portion of pancreas with FAT
What clinical symptoms diagnose CF
frequent pulm infections (pseudomonas)
Diarrhea, malasb (steatorrhea), and failure to thrive
Dehydration (freq episones)
testing used to diagnose CF
sweat Cl- test
PFTs
Genetic Testing
How is CF managed
Daily Pulm Toilet/Inhalers that loosen secretions
high dose pancreatic enzymes
treat pulm infections
what is the pathophys of the failure to thrive seen in CF?
no acinar cells = no pancreatic digestive enzymes
most common cyst-like lesion in the pancrease
pseudocyst
In general, of the neoplastic cysts __ _have very little malignant potential while ___ have malignant potential
serous = not malignant mucinous = malignant
what are 4 examples of cystic neoplasms of the pancreas. which are benign and malignant?
Benign:
Serious cystic tumors
Malignant:
Mucinous Cyst neoplasms
Intraductal Papillary mucinous neoplasms
solid pseudopapillary Neoplasms
CEA is a tumor marker for
mucinous cystic neoplasm
ERCP is a tumor marker for
intraductal papillary mucinous neoplasms
common presentation of pancreatic cancer
weight loss, abdominal pain, jaundice
90% of pancreatic cancers are
ductal adenocarcinomas
What are the miscellaneous pancreatic cancers that account for the other 10%
cystic neoplasms
acinar cell neoplasms
lymphomas and sarcomas
neuroendocrine tumors (gastrinoma, insulinomas, glucagonomas)
not going into the histology
nichols made it sound like even pathologists struggle with it
Risk factors for pancreatic cancer
smoking
alcohol in the setting on chronic pancreatitis
hereditary pancreatitis
family history of pancreatic cancer
What is the tumor marker for pancreatic adenocarcinoma
CA19-9 **DUCTAL cell tumor marker
pathophys of jaundice in pancreatic adenocarcinoma
obstruction of CBD
What clinical presentation is a clue that the pt may have a mass in the head of their pancreas
painless jaundice
**60-70% of pancreatic adenocarcinomas are in the head of the pancreas
what surgical options can cure some pancreatic cancers?
distal pancreatectomy (tail lesions) Whipple resection (for head lesions)
**chemo and radiation have limited effectiveness therefore surgery is the only chance for a cure
what is the best prognostic factor for pancreatic cancers
retroperitoneal resection margin
**only for resectable adenocarcinomas in the head of the pancreas
