B5.028 GI Cancers- Non Tubular Flashcards
HCC risk factors
often arises in cirrhotic livers, only 10% non cirrhotic
-HBV present in many non-cirrhotic patients
risk factors:
-hep B and C
-alcohol
-environmental
HCC symptoms/presentation
- often no symptoms
- ill-defined upper abdominal pain, fatigue, weight loss, abdominal mass/hepatomegaly
- jaundice if biliary obstruction present
- often older age
work up for HCC
labs: may show elevated serum AFP ( in 50%)
imaging: CT/MRI with vascular contrast often diagnostic
staging of HCC
T(tumor): size, number, vascular invasion, invasion of adjacent structures
N (lymph nodes)
M (metastasis)
treatment of HCC
surgical resection (if possible, not usually possible in cirrhotic liver) ablation, chemoembolization, chemotherapy
prognosis of HCC
small tumors (<2 cm) have a good prognosis with possibility for cure large tumors have a poor prognosis and average survival of 2 years
molecular features of HCC
- activation of beta-catenin (40%)
- inactivation of p53 (60%), prominent in tumors associated with aflatoxin exposure
HCC precursor lesions
non-cirrhotic livers: -small cell change -large cell change cirrhotic livers: -dysplastic nodules -small cell change
what do you do if you find an HCC precursor lesion?
surveillance cancer screening
differential of cirrhosis with large nodule
macroregenerative nodule
dysplastic nodule
HCC
features of large cell change
large hepatocytes with large, often atypical nuclei scattered among normal size hepatocytes with round, typical nuclei
features of small cell change
abnormal cells have a high nuclear-to-cytoplasmic ratio and are separated by thickened plates
cellular atypia
increased nuclear to cytoplasmic ratio
distorted architecture
thickened call plates
bile production (no mucin)
fibrolamellar carcinoma HCC variant description
clinical and labs: -young patients, 5-35 -non-cirrhotic liver -almost always negative for AFP gross appearance: -firm with fibrous band running through tumor -central scar
microscopic appearance of fibrolamellar carcinoma
hepatocytes with lots of mitochondria, giving a pink “oncocytic” appearance
- growth is nested or cord like pattern
- dense collagen fibers is the hallmark
what is cholangiocarcinoma
cancer arising from biliary tree/bile duct
-can arise in any area: intrahepatic or extrahepatic
risk factors for cholangiocarcinoma
chronic inflammation, cholestasis
- liver flukes
- chronic inflammatory conditions
clinical presentation of cholangiocarcinoma
asymptomatic
symptoms of biliary obstruction of liver mass
location of cholangiocarcinomas
intrahepatic- 10%
perihilar- 50-60%
distal- 20-30%
gross appearance
tan-white solid nodule, may be multiple
non-cirrhotic liver
microscopic appearance of cholangiocarcinoma
adenocarcinoma (forms glands, produces mucin)
cells with enlarged nuclear to cytoplasmic ratio, glands are angulated instead of round
precursor lesions of cholangiocarcinoma
dysplasia of normal bile ducts
BilIN (biliary intraepithelial neoplasia), grade 1-3
some cystic neoplasms
cholangiocarcinoma staging
depends on location of tumor (intrahepatic, perihilar, distal)
size, vascular invasion, invasion of adjacent structures
cholangiocarcinoma treatment
surgical resection
cholangiocarcinoma prognosis
poor, 15% survival at 2 years
liver metastasis
most common cause of liver tumor
usually multiple nodules, can be very large before symptoms occur
colon, lung, breast, pancreas most common primary sites for spread to liver (most are adenocarcinomas)
pancreatic acinar cells
exocrine function
secrete enzymes for digestion
islets of Langerhans
endocrine function
insulin, glucagon, somatostatin secretion
characteristics of pancreatic ductal adenocarcinoma
most common pancreatic cancer
4th leading cause of cancer deaths
one of the highest mortality rates of any cancer
pancreatic ductal adenocarcinoma risk factors
cigarette smoking
chronic pancreatitis
diabetes
familial BRCA2 and CDKN2A mutations
pancreatic ductal adenocarcinoma clinical presentation
older individuals (60-80) usually asymptomatic may have features of biliary obstruction
pancreatic ductal adenocarcinoma locations
head - 60%
body - 15%
tail- 5%
entire gland - 20%
pancreatic ductal adenocarcinoma pathogenesis
multiple molecular alterations occurs early alterations -telomere shortening (almost all tumors) -KRAS mutations (90%) later alterations -mutation and inactivation of many genes
pancreatic ductal adenocarcinoma gross appearance
tan-white, firm mass
usually singular
microscopic appearance of pancreatic ductal adenocarcinoma
adenocarcinoma = proliferation of atypical glands with mucin production
densely fibrotic stroma
inflammatory cells
precursor lesions of pancreatic ductal adenocarcinoma
dysplasia of the pancreatic ducts
-PanIN (pancreatic intraepithelial neoplasia) 1-3
molecular alterations occur in the precursor lesions
treatment for pancreatic ductal adenocarcinoma
resection if possible and without metastasis
staging of pancreatic ductal adenocarcinoma
based on size, invasion of large arteries (celiac axis, superior mesenteric artery, common hepatic artery)
describe pancreatic neuroendocrine tumors
less common than ductal adenocarcinomas (only 2% of pancreas tumors)
may occur anywhere in the pancreas
can be benign or malignant
types of pancreatic neuroendocrine tumors
may be functional or non-functional
- in pancreas, neuroendocrine cells secrete hormones
- insulin, gastrin, somatostatin, glucagon, VIP
- 90% of insulinomas are benign
- 60-90% of non-insulinomas are malignant
features of insulinoma
secrete insulin symptoms of hypoglycemic episodes labs: high insulin, low glucose treatment: resection benign in 90%
features of gastrinoma
secrete gastrin (Zollinger-Ellison syndrome)
-stimulated parietal cells in the stomach to produce acid
symptoms of hypersecretion of gastric acid and severe peptic ulceration
treatment: resection
nonfunctional neuroendocrine tumors
no hormone secretion
usually asymptomatic, unless blocking bile ducts
treatment: resection
gross appearance of pancreatic neuroendocrine tumors
similar for functional and non-functional
solid, tan-red nodule
pancreatic neuroendocrine tumor histology
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)
positive for chromogranin stain
pancreatic neuroendocrine tumor treatment and staging
treatment: resection
staging: based on size, invasion to adjacent structures
