B5.028 - GI Cancers, Non Tubular

Question 1

Answer 1

normal liver

Question 2

liver cancer locations

Answer 2

hepatocyte - hepatocellular carcinoma, fibrolamellar carcinoma

bile ducts - cholangiocarcinoma

blood vessels

metastasis

Question 3

features of hepatocellular carcinoma and risk factors

Answer 3

often arises in cirrhotic livers

risk factors:

viral hepatitis

alcohol

environmental (aflatoxin)

Question 4

symptoms of hepatocellular carcinoma

Answer 4

often no symptoms

ill defined upper abdominal pain, fatigue, weight loss, hepatmegaly

jaundice if biliary obstruction

Question 5

work up for hepatocellular carcinoma

Answer 5

may show elevated AFP ~50%

CT/MRI with vascular contrast often diagnostic

Question 6

staging of HCC

Answer 6

T - size, number, vascular invasion

N - lymph nodes

M - metastasis

Question 7

treatment of HCC

Answer 7

surgican resection

ablation, chemoembolization, chemo

Question 8

prognosis of HCC

Answer 8

small tumors <2cm have good prognosis

large have poor prognosis and avg survival 2 yrs

Question 9

molecular features of HCC

Answer 9

activation of beta catenin

inactivation of p53 - prominent in tumors with aflatoxin exposure

Question 10

HCC precursor lesions

Answer 10

non cirrhotic

* small cell change

large cell change (not direct)

cirrhotic

* dysplastic nodules

* small cell change

Question 11

Answer 11

cirrhosis with large nodule, precursor lesion

differential includes macrogenerative nodule, dyplastic nodule, HCC

Question 12

Answer 12

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

Question 13

Answer 13

cellular atypia

HCC

increased nuclear to cytoplasmic ration

distorted architecture

thickened cell plates

bile production (no mucin)

Question 14

Answer 14

•Cellular atypia

–Increased nuclear to cytoplasmic ratio

–Distorted architecture

–Thickened cell plates (more than two cells thick)

–Bile production (no mucin)

Question 15

fibrolamellar carcinoma

Answer 15

different clinical presentation, histology and molecular changes

young patients, 5-35

non cirrhotic liver

almost always negative for serum AFP

Question 16

fibrolamellar carcinoma gross appearance

Answer 16

firm with fibrous bands running through tumor

central scar

Question 17

Answer 17

fibrolamellar carcinoma

Question 18

Answer 18

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

Question 19

Answer 19

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

Question 20

features of cholangiocarcinoma

Answer 20

cancer arising from biliary tree/bile duct

Question 21

risk factors for cholangiocarcinoma and clinical presentation

Answer 21

chronic inflammation, cholestasis

liver flukes, chronic inflammation

asymptomatic, sympotoms of biliary obstruction or liver mass

Question 22

Answer 22

cholangiocarcinoma

Question 23

gross appearance of cholangiocarcinoma

Answer 23

tan white solid nodule; may be multiple

non cirrhotic liver

Question 24

Answer 24

cholangiocarcinoma

•Microscopic

–Adenocarcinoma

•Forms glands, produces mucin

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

Question 25

Answer 25

cholangiocarcinoma ## Footnote •Microscopic –Adenocarcinoma •Forms glands, **produces mucin** –Cells with enlarged nuclear to cytoplasmic ratio, glands are angulated instead of round

Question 26

precursor of cholangiocarcinoma

Answer 26

precursor lesion is dysplasia of normal bile ducts BilIN - grade 1,2,3 some cystic neoplasms

Question 27

staging of cholangiocarcinoma

Answer 27

depends on location of tumor, size, invasion

Question 28

treatment of cholangiocarcinoma

Answer 28

surgical resection

Question 29

prognosis of cholangiocarcinoma

Answer 29

poor, 15% at 2 years

Question 30

liver tumors usually caused by

Answer 30

metastasis usually multiple nodules cna be very large before symptoms occur colon, lung, breast, pancrease most common primart sites

Question 31

most liver metastasis are

Answer 31

adenocarcinomas (glandular microscopic shape, mucin production

Question 32

acinar cells

Answer 32

exocrine function secrete enzymes for digestion

Question 33

islet of langerhans

Answer 33

endocrine function insulin, glucagon, somatostatin secretion

Question 34

tumor types of the pancreas

Answer 34

ducta cells - pancreatic adenocarcinoma islets of langerhans - neuroendocrine tumors

Question 35

features of pancreatic ductal adenocarcinoma

Answer 35

most common cancer of the pancreas 4th leading cause of cancer deaths

Question 36

risk factors for pancreatic ductal adenocarcinoma

Answer 36

cigarettes chronic pancreatitis diabetes familial BRCA2, CDKN2A

Question 37

location of pancreatic ductal adenocarcarcinoma

Answer 37

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

Question 38

molecular pathogenesis of pancreatic ductal adenocarcinoma

Answer 38

multiple molecular alterations occur early alterations - telomere shortening, KRAS mutations later - mutation and inactivation of many genes

Question 39

gross and microscopic appearance of pancreatic ductal adenocarcinoma

Answer 39

gross - tan-white, firm mass, usually singular micro - proliferation of atypical glands with mucin production

Question 40

Answer 40

carcinoma of the pancreas

Question 41

Answer 41

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)

Question 42

precursors of pancreatic duct adenocarcinoma

Answer 42

dysplasia of pancreatic ducts - PanIN

Question 43

Answer 43

pancreatic intraepithelial neoplasia grade 3 involving small pancreatic duct

Question 44

treatment of pancreatic ductal adenocarcinoma

Answer 44

resection

Question 45

staging of pancreatic duct adenocarcinoma

Answer 45

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

Question 46

describe pancreatic neuroendocrine tumors

Answer 46

* 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

Question 47

what type of PanNET are most common

Answer 47

insulinomas, most are benign (90%) 60-90% non insulinomas are malignant

Question 48

features of insulinomas, treatment, labs, symptoms

Answer 48

•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%

Question 49

describe gastrinomas (treatment, symptoms, features)

Answer 49

•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

Question 50

non functional neuroendocrine tumors

Answer 50

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

Question 51

gross and histo of pancreatic endocrine tumors

Answer 51

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

Question 52

Answer 52

a well circumscribed solid mass in pancreas is typical fro low grade neuroendocrine tumors

Question 53

Answer 53

neuroendocrine tumor: nested and corded growth pattern, Nuclei have stippled chromatin "salt and pepper"

Question 54

Answer 54

pancreatic neuroendocrine tumor: solid growth, salt and pepper chromatin

Question 55

Answer 55

diffuse positive staining for chromogranin is shown. neuroendocrine

Question 56

treatment/staging of pancreatic neuroendocrine tumors

Answer 56

resection staging based on size, invasion of adjacent structures