B5-028 GI Cancers, Non-Tubular Flashcards
a tumor in the head of the pancreas is likely to involve the […]
biliary duct
normal pancreas
pancreatic adenocarcinomas arise from
ductal cells
pacreatic NETs arise from
islets of Langerhans
- most common cancer of the pancreas
- third leading cause of cancer deaths
pancreatic ductal adenocarcinoma
risk factors for pancreatic ductal carcinoma
- smoking
- chronic pancreatitis
- diabetes
- BRCA2 and CDKN2A mutations
- older individuals
- back pain, unexplained weight loss, fatigue
- jaundice
- new onset diabetes
pancreatic ductal carcinoma
pancreatic ductal carcinoma in the […] of the pancreas tend to present at a more advanced stage
tail/body
most are in head
mutations seen in pathogenesis of pancreatic ductal carcinoma
early: **KRAS **
later: SMAD4
pancreatic ductal carcinoma
proliferation of malignant glands with or without mucin production
pancreatic ductal carcinoma
fine needle biopsy of pancreatic ductal carcinoma
intital diagnosis by fine needle biopsy
- precursor lesion
- telomere shortening, KRAS mutation (low grade)
- later, CDKN2A mutation (high grade)
pancreatic intraepithelial neoplasia
PanIN
high grade PanIN
2 types of cystic precursor lesions to pancreatic ductal carcinoma
- intraductal papillary mucinous neoplasm
- mucinous cystic neoplasm
always always curable early on
main pancreatic duct dilated and filled with mucin
intraductal papillary mucinous neoplasm
papillary structures lined by mucinous epithelium
intraductal papillary mucinous neoplasm
- majority occur in pancreatic head
- KRAS and GNAS mutations
- more common in men
- can be multifocal
intraductal papillay mucinous neoplasms
mucinous cystic neoplasm
mucinous cystic neoplasm
- more common in females
- occurs in body/tail
- KRAS mutations
- no connection to ductal system
mucinous cystic neoplasm
tumors associated with clinical syndromes caused by abnormal secretion of hormones by the tumor
functional PanNETs
insulinomas, gastrinomas, glucagonomas, somatostatinomas, VIPomas
functional PanNETs
may secrete peptide or biogenic substance at levels insufficient to cause symptoms or do not give rise to a clinical syndrome
non-functional PanNETs
incidentally discovered or become clinically apparent due to size, invasion or metastasis
non-functional PanNETs
tumors not associated with a clinical hormone hypersecretion syndrome
non-functional PanNETs
cause hyperinsulinemia and hypoglycemia
insulinoma
functional PanNETs
- palpitations, tremor
- sweating, hunger, paraesthesia
- severe weakness, psychiatric, and neurological manifestations
insulinoma
functional PanNETs
deposits of amyloid in between stroma
insulinoma
functional PanNETs
whipple triad:
hypoglycemia
low plasma glucose
symptom relief after glucose administration
insulinoma
functional PanNETs
best diagnostic test for insulinoma
prolonged fasting measurement of blood glucose, insulin, C-peptide, and pro insulin
gastrinomas are more common in the […] than the pancreas
duodenum
- leads to peptic ulceration, ZE
- typical medical management of high gastrin levels does not work
gastrinoma
functional PanNETs
- usually asymptomatic unless blocking bile ducts
- 55-75% behave in a malignant manner
non-functional PanNETs
non-functional PanNETs
MEN1 mutations increase the risk of
NETs
pancreatic polypeptide secreting NETs are more commonly associated with a mutation in
MEN1
solid, tan-red nodules
pancreatic NETs
“salt and pepper” granular chromatin in uniform cells
NETs
treatment for NETs
octreotide
resection
- common in women of childbearing age
- linked to OCPs and anabolic steroids
- often asymptomatic, may cause abdominal pain
hepatocellular adenoma
hepatocellular adenoma are […] and arise in non-cirrhotic livers
benign
- strong female predilection
- LOF mutation in trancription factor that regulates many genes in hepatocytes and pancreatic islets
- low risk of transformation to HCC
HNF1a-inactivated hepatocellular adenoma
- commonly associated with obesity and metabolic syndrome
- leads to constitutive JAK/STAT signaling
- low risk of HCC
inflammatory hepatocellular adenoma
- activating mutation in gene
- up to 40% in men
- high risk for malignant transformation
- type of hepatocellular adenoma
b-catenin activated hepatocellular adenoma
positive on B-catenin IHC
most common subtype of hepatocellular adenoma
inflammatory
least common subtype of hepatocellular adenoma
B-catenin
hepatocellular adenoma
hepatocellular adenoma
if hepatocellular adenoma is larger than 5 cm…
risk for hemorrhage
consider resection
if smaller, watchful waiting
treatment for B-catenin hepatocellular adenoma
resect
- young adult women
- associated with Bud-Chiari syndrome, hemangiomas
- usually asymptomatic, cause abdominal pain
- non-neoplastic
Focal Nodular Hyperplasia
focal nodular hyperplasia
focal nodular hyperplasia
central stellate scar
thick walled arteries
focal nodular hyperplasia
most common benign tumor of the liver
cavernous hemangioma
- discrete nodule <5 cm
- dilated, thin vessel walls
cavernous hemangioma
cavernous hemangioma
cavernous hemangioma
most common cause of liver tumors
metastasis
most common cancers that metastasize to liver
6
- colorectal
- stomach
- breast
- pancreas
- lung
- melanoma
shows GNAS and KRAS mutations
intraductal papillary mucinous neoplasm
localized vascular malformation leading to mass forming hyperplastic response of hepatocytes
focal nodular neoplasia
are primary liver tumors common?
no
metastases to the liver are more common
most common primary tumor to spread to the liver
6
- colon
- rectum
- breast
- stomach
- pancreas
- melanoma
[…] occurs early in all pancreatic cancer and is thought to lead to multiple chromosomal abnormalities
telomere shortening
