B5.028 GI Cancers- Non Tubular

Question 1

HCC risk factors

Answer 1

often arises in cirrhotic livers, only 10% non cirrhotic
-HBV present in many non-cirrhotic patients
risk factors:
-hep B and C
-alcohol
-environmental

Question 2

HCC symptoms/presentation

Answer 2

• often no symptoms
• ill-defined upper abdominal pain, fatigue, weight loss, abdominal mass/hepatomegaly
• jaundice if biliary obstruction present
• often older age

Question 3

work up for HCC

Answer 3

labs: may show elevated serum AFP ( in 50%)
imaging: CT/MRI with vascular contrast often diagnostic

Question 4

staging of HCC

Answer 4

T(tumor): size, number, vascular invasion, invasion of adjacent structures
N (lymph nodes)
M (metastasis)

Question 5

treatment of HCC

Answer 5

surgical resection (if possible, not usually possible in cirrhotic liver)
ablation, chemoembolization, chemotherapy

Question 6

prognosis of HCC

Answer 6

small tumors (<2 cm) have a good prognosis with possibility for cure
large tumors have a poor prognosis and average survival of 2 years

Question 7

molecular features of HCC

Answer 7

• activation of beta-catenin (40%)

- inactivation of p53 (60%), prominent in tumors associated with aflatoxin exposure

Question 8

HCC precursor lesions

Answer 8

non-cirrhotic livers:
-small cell change
-large cell change
cirrhotic livers:
-dysplastic nodules
-small cell change

Question 9

what do you do if you find an HCC precursor lesion?

Answer 9

surveillance cancer screening

Question 10

differential of cirrhosis with large nodule

Answer 10

macroregenerative nodule
dysplastic nodule
HCC

Question 11

features of large cell change

Answer 11

large hepatocytes with large, often atypical nuclei scattered among normal size hepatocytes with round, typical nuclei

Question 12

features of small cell change

Answer 12

abnormal cells have a high nuclear-to-cytoplasmic ratio and are separated by thickened plates

Question 13

cellular atypia

Answer 13

increased nuclear to cytoplasmic ratio
distorted architecture
thickened call plates
bile production (no mucin)

Question 14

fibrolamellar carcinoma HCC variant description

Answer 14

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

Question 15

microscopic appearance of fibrolamellar carcinoma

Answer 15

hepatocytes with lots of mitochondria, giving a pink “oncocytic” appearance

• growth is nested or cord like pattern
• dense collagen fibers is the hallmark

Question 16

what is cholangiocarcinoma

Answer 16

cancer arising from biliary tree/bile duct

-can arise in any area: intrahepatic or extrahepatic

Question 17

risk factors for cholangiocarcinoma

Answer 17

chronic inflammation, cholestasis

• liver flukes
• chronic inflammatory conditions

Question 18

clinical presentation of cholangiocarcinoma

Answer 18

asymptomatic

symptoms of biliary obstruction of liver mass

Question 19

location of cholangiocarcinomas

Answer 19

intrahepatic- 10%
perihilar- 50-60%
distal- 20-30%

Question 20

gross appearance

Answer 20

tan-white solid nodule, may be multiple

non-cirrhotic liver

Question 21

microscopic appearance of cholangiocarcinoma

Answer 21

adenocarcinoma (forms glands, produces mucin)

cells with enlarged nuclear to cytoplasmic ratio, glands are angulated instead of round

Question 22

precursor lesions of cholangiocarcinoma

Answer 22

dysplasia of normal bile ducts
BilIN (biliary intraepithelial neoplasia), grade 1-3
some cystic neoplasms

Question 23

cholangiocarcinoma staging

Answer 23

depends on location of tumor (intrahepatic, perihilar, distal)
size, vascular invasion, invasion of adjacent structures

Question 24

cholangiocarcinoma treatment

Answer 24

surgical resection

Question 25

cholangiocarcinoma prognosis

Answer 25

poor, 15% survival at 2 years

Question 26

liver metastasis

Answer 26

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)

Question 27

pancreatic acinar cells

Answer 27

exocrine function | secrete enzymes for digestion

Question 28

islets of Langerhans

Answer 28

endocrine function | insulin, glucagon, somatostatin secretion

Question 29

characteristics of pancreatic ductal adenocarcinoma

Answer 29

most common pancreatic cancer 4th leading cause of cancer deaths one of the highest mortality rates of any cancer

Question 30

pancreatic ductal adenocarcinoma risk factors

Answer 30

cigarette smoking chronic pancreatitis diabetes familial BRCA2 and CDKN2A mutations

Question 31

pancreatic ductal adenocarcinoma clinical presentation

Answer 31

``` older individuals (60-80) usually asymptomatic may have features of biliary obstruction ```

Question 32

pancreatic ductal adenocarcinoma locations

Answer 32

head - 60% body - 15% tail- 5% entire gland - 20%

Question 33

pancreatic ductal adenocarcinoma pathogenesis

Answer 33

``` multiple molecular alterations occurs early alterations -telomere shortening (almost all tumors) -KRAS mutations (90%) later alterations -mutation and inactivation of many genes ```

Question 34

pancreatic ductal adenocarcinoma gross appearance

Answer 34

tan-white, firm mass | usually singular

Question 35

microscopic appearance of pancreatic ductal adenocarcinoma

Answer 35

adenocarcinoma = proliferation of atypical glands with mucin production densely fibrotic stroma inflammatory cells

Question 36

precursor lesions of pancreatic ductal adenocarcinoma

Answer 36

dysplasia of the pancreatic ducts -PanIN (pancreatic intraepithelial neoplasia) 1-3 molecular alterations occur in the precursor lesions

Question 37

treatment for pancreatic ductal adenocarcinoma

Answer 37

resection if possible and without metastasis

Question 38

staging of pancreatic ductal adenocarcinoma

Answer 38

based on size, invasion of large arteries (celiac axis, superior mesenteric artery, common hepatic artery)

Question 39

describe pancreatic neuroendocrine tumors

Answer 39

less common than ductal adenocarcinomas (only 2% of pancreas tumors) may occur anywhere in the pancreas can be benign or malignant

Question 40

types of pancreatic neuroendocrine tumors

Answer 40

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

Question 41

features of insulinoma

Answer 41

``` secrete insulin symptoms of hypoglycemic episodes labs: high insulin, low glucose treatment: resection benign in 90% ```

Question 42

features of gastrinoma

Answer 42

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

Question 43

nonfunctional neuroendocrine tumors

Answer 43

no hormone secretion usually asymptomatic, unless blocking bile ducts treatment: resection

Question 44

gross appearance of pancreatic neuroendocrine tumors

Answer 44

similar for functional and non-functional | solid, tan-red nodule

Question 45

pancreatic neuroendocrine tumor histology

Answer 45

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

Question 46

pancreatic neuroendocrine tumor treatment and staging

Answer 46

treatment: resection staging: based on size, invasion to adjacent structures