Pancreatitis 2 Flashcards
What is the cause of congenital cysts?
most likely due to anomalous pancreatic duct development
Describe the make up of congenital cysts.
Unilocular, thin-walled cysts with a thin, fibrous capsule filled by clear serous fluid
Congenital cysts may be sporadic but they can also be part of what inherited conditions?
Autosomal-dominant polycystic kidney disease
von Hippel-Lindau disease
What are autosomal-dominant polycystic kidney diseases?
cysts in liver, kidney and pancreas
What is von Hippel-Lindau disease?
vascular neoplasms are found in the retina and cerebellum or brain stem in association with congenital cysts (and also neoplasms) in the pancreas, liver, and kidney
When are pseudocysts formed and what are they caused by?
Formed when areas of intrapancreatic or peripancreatic hemorrhagic fat necrosis caused by acute pancreatitis or traumatic injury are walled off by fibrosis and granulation tissue
Do pseudocyst have an epithelial lining?
no
What are pseudocysts lined by instead of epithelial lining?
fibrin and granulation tissue
Where are serous cyst adenomas mostly found?
tail of pancreas
How would you treat serous cystadenomas?
surgical resection
Describe the make up of serous cystadenomas.
Glycogen-rich cuboidal cells surrounding small cysts containing clear, straw-colored fluid
What are mucinous cystic neoplasms?
precursors to invasive carcinomas, painless, slow-growing masses arising in the tail
What are the cysts in muscinous cystic neoplasms lined by (type of epithelium)? and what are they filled with?
cysts are filled with thick, tenacious mucin, and lined by a columnar mucinous epithelium with an associated densely
cellular stroma (resembles ovarian stroma), that expresses ER/PR receptors and Inhibin
What type of mutation are linked to mucinous cystic neoplasms?
- Kras mutations in in 50% cases, 1/3rd cases will harbor an invasive adenocarcinoma with TP53 and SMAD4 mutations
What are intra-ductal papillary mucinous neoplasms?
Cysts that ontain mucin, lack dense “ovarian” stroma (unlike mucinous cystic)
Are Intra-ductal papillary mucinous neoplasm benign or malignant?
- Can be benign, borderline malignant, or malignant
What type of mutations are associated with Intra-ductal papillary mucinous neoplasm?
KRAS mutations in 80% of tumors and loss of-function RNF43 mutations in up to 50%, TP53 and SMAD4 mutations typically occur only with transition to invasive cancer, oncogenic mutations of GNAS in 2/3rd of IPMNs
Where can intra-ductal papillary mucinous neoplasms be found?
involve larger ducts mostly in the head of the pancreas
Be able to label this diagram to understand most common locations in pathologies related to the pancreas.
Label using slide created in goodnotes.
What is solid pseudo-papillary neoplasm?
type of low grade malignant neoplasm with large, well-circumscribed masses with solid and cystic components
What are the related mutations in solid pseudo-papillary neoplasms?
- Β-catenin/adenomatous polyposis coli genetic pathway is altered due to activating mutations of β-catenin
What is the best treatment for solid pseudo-papillary neoplasm?
surgical resection
What is the strongest environmental influence in getting pancreatic cancer?
smoking
Familial pancreatic cancer can be linked to what type of mutations?
BRCA2 mutations
Can there be a greater risk of pancreatic cancer in individuals with visceral obesity and high body mass index?
yes
What is another name for antecedent lesions on pancreatic cancer?
“pancreatic intraepithelial neoplasias” (PanINs)
What is the most frequently altered oncogene in pancreatic cancer?
K-RAS
Understand and be able to explain the model for the development of pancreatic cancer.
- Telomere shortening, and mutations of oncogene K-RAS at early stages
- Inactivation of the CDKN2A tumor suppressor gene that encodes the cell cycle regulator p16 at intermediate stages
- Inactivation of p53, SMAD4, and BRCA2 tumor suppressor genes at late stages
Describe the gross appearance of the pancreas in those with pancreatic carcinoma.
- Gross: hard, stellate, gray-white, poorly defined masses
Describe the microscopic appearance of the pancreas in a person with pancreatic carcinoma
poorly formed malignant glands, desmoplastic response, perineural invasion
What is a Whipple procedure?
En bloc resection of the pancreatic head and neck (distal pancreas remains to prevent diabetes mellitus) and resection of part of CBD
In some cases, resection of the antrum with vagotomy
What is considered the marker for pancreatic cancer?
CA19-9
Are CEA and CA19-9 specific or sensitive enough to be used as a screening test for pancreatic cancer?
no
Are CEA and CA19-9 levels increased or decreased in pancreatic caner?
elevated/ increased
Does the US Screening and Prevention Task Force (USPSTF) currently recommend screening the general population for pancreatic cancer?
no
