B5.028 - GI Cancers, Non Tubular Flashcards
normal liver
liver cancer locations
hepatocyte - hepatocellular carcinoma, fibrolamellar carcinoma
bile ducts - cholangiocarcinoma
blood vessels
metastasis
features of hepatocellular carcinoma and risk factors
often arises in cirrhotic livers
risk factors:
viral hepatitis
alcohol
environmental (aflatoxin)
symptoms of hepatocellular carcinoma
often no symptoms
ill defined upper abdominal pain, fatigue, weight loss, hepatmegaly
jaundice if biliary obstruction
work up for hepatocellular carcinoma
may show elevated AFP ~50%
CT/MRI with vascular contrast often diagnostic
staging of HCC
T - size, number, vascular invasion
N - lymph nodes
M - metastasis
treatment of HCC
surgican resection
ablation, chemoembolization, chemo
prognosis of HCC
small tumors <2cm have good prognosis
large have poor prognosis and avg survival 2 yrs
molecular features of HCC
activation of beta catenin
inactivation of p53 - prominent in tumors with aflatoxin exposure
HCC precursor lesions
non cirrhotic
* small cell change
large cell change (not direct)
cirrhotic
* dysplastic nodules
* small cell change
cirrhosis with large nodule, precursor lesion
differential includes macrogenerative nodule, dyplastic nodule, HCC
top:
large cell change, large atypical nuclei are scattered among normal size hepatocytes
bottom:
small cell change, abnormal cells have high nuclear to cytoplasmic ration separated by thickened plates
cellular atypia
HCC
increased nuclear to cytoplasmic ration
distorted architecture
thickened cell plates
bile production (no mucin)
•Cellular atypia
–Increased nuclear to cytoplasmic ratio
–Distorted architecture
–Thickened cell plates (more than two cells thick)
–Bile production (no mucin)
fibrolamellar carcinoma
different clinical presentation, histology and molecular changes
young patients, 5-35
non cirrhotic liver
almost always negative for serum AFP
fibrolamellar carcinoma gross appearance
firm with fibrous bands running through tumor
central scar
fibrolamellar carcinoma
fibrolamellar carcinoma
microscopic
* hepatocytes with lots of mitochondira, giving a pink oncocytic appearance
* growth is in nested or cord like pattern
* dense collagen fibers is the hallmark
fibrolamellar carcinoma
•Microscopic
–Hepatocytes with lots of mitochondria, giving a pink “oncocytic” appearance
–Growth is in nested or cord-like pattern
–Dense collagen fibers is the hallmark
features of cholangiocarcinoma
cancer arising from biliary tree/bile duct
risk factors for cholangiocarcinoma and clinical presentation
chronic inflammation, cholestasis
liver flukes, chronic inflammation
asymptomatic, sympotoms of biliary obstruction or liver mass
cholangiocarcinoma
gross appearance of cholangiocarcinoma
tan white solid nodule; may be multiple
non cirrhotic liver
cholangiocarcinoma
•Microscopic
–Adenocarcinoma
•Forms glands, produces mucin
–Cells with enlarged nuclear to cytoplasmic ratio, glands are angulated instead of round
cholangiocarcinoma
•Microscopic
–Adenocarcinoma
•Forms glands, produces mucin
–Cells with enlarged nuclear to cytoplasmic ratio, glands are angulated instead of round
precursor of cholangiocarcinoma
precursor lesion is dysplasia of normal bile ducts
BilIN - grade 1,2,3
some cystic neoplasms
staging of cholangiocarcinoma
depends on location of tumor, size, invasion
treatment of cholangiocarcinoma
surgical resection
prognosis of cholangiocarcinoma
poor, 15% at 2 years
liver tumors usually caused by
metastasis
usually multiple nodules
cna be very large before symptoms occur
colon, lung, breast, pancrease most common primart sites
most liver metastasis are
adenocarcinomas (glandular microscopic shape, mucin production
acinar cells
exocrine function
secrete enzymes for digestion
islet of langerhans
endocrine function
insulin, glucagon, somatostatin secretion
tumor types of the pancreas
ducta cells - pancreatic adenocarcinoma
islets of langerhans - neuroendocrine tumors
features of pancreatic ductal adenocarcinoma
most common cancer of the pancreas
4th leading cause of cancer deaths
risk factors for pancreatic ductal adenocarcinoma
cigarettes
chronic pancreatitis
diabetes
familial BRCA2, CDKN2A
location of pancreatic ductal adenocarcarcinoma
head of pancreas - 60%
body - 15%
tail - 5%
entire gland - 20%
molecular pathogenesis of pancreatic ductal adenocarcinoma
multiple molecular alterations occur
early alterations - telomere shortening, KRAS mutations
later - mutation and inactivation of many genes
gross and microscopic appearance of pancreatic ductal adenocarcinoma
gross - tan-white, firm mass, usually singular
micro - proliferation of atypical glands with mucin production
carcinoma of the pancreas
Carcinoma of the pancreas. Poorly formed glands are present in densely fibrotic stroma within the pancreatic substance; some inflammatory cells are also present. The cells would be positive for mucin (adenocarcinoma)
precursors of pancreatic duct adenocarcinoma
dysplasia of pancreatic ducts - PanIN
pancreatic intraepithelial neoplasia grade 3 involving small pancreatic duct
treatment of pancreatic ductal adenocarcinoma
resection
staging of pancreatic duct adenocarcinoma
based on size, invasion of large arteries (celiac axis, superior mesenteric artery, common hepatic artery)
describe pancreatic neuroendocrine tumors
- Pancreatic neuroendocrine tumors (PanNETs) are less common than pancreatic ductal adenocarcinoma (2% of pancreas tumors)
- May occur anywhere in the pancreas
–Single or multiple tumors possible
•Can be benign or malignant
what type of PanNET are most common
insulinomas, most are benign (90%)
60-90% non insulinomas are malignant
features of insulinomas, treatment, labs, symptoms
•Insulinoma
–Secrete insulin
–Symptoms of hypoglycemic episodes (blood glucose <50mg/dL)
- Confusion, stupor, loss of consciousness
- Treat by giving food, parenteral glucose
–Labs: high insulin, low glucose
–Treatment: resection
–Benign in 90%
describe gastrinomas (treatment, symptoms, features)
•Gastrinoma
–Secrete gastrin (Zollinger-Ellison syndrome)
•Stimulates parietal cells in the stomach to produce acid
–Symptoms of hypersecretion of gastric acid and severe peptic ulceration
•Typical medical management does not work
–Treatment: Resection
non functional neuroendocrine tumors
no hormone secretion
usually asymptomatic, unless blocking bile ducts
treatment: resection
gross and histo of pancreatic endocrine tumors
gross - similar for functional and non functional, solid tan-red nodule
histo - similar for functional and non functional
* architectural patterns, nested, cords, solid
* cells - uniform cells, moderate amount of cytoplasm, nuclei with granular chromatin “salt and pepper”
* posiitve for chromogranin stain
a well circumscribed solid mass in pancreas is typical fro low grade neuroendocrine tumors
neuroendocrine tumor: nested and corded growth pattern, Nuclei have stippled chromatin “salt and pepper”
pancreatic neuroendocrine tumor: solid growth, salt and pepper chromatin
diffuse positive staining for chromogranin is shown. neuroendocrine
treatment/staging of pancreatic neuroendocrine tumors
resection
staging based on size, invasion of adjacent structures
