Neoplastic States of Pancreas/Gallbladder

Question 1

What are two vessels below neck of pancreas?

Answer 1

SMA and SMV

Question 2

What are three important histological components of pancreas?

Answer 2

Acini
Islets
Ducts

Question 3

What are two broad categories of pancreas lesions?

Answer 3

Cystic and Non-cystic

Question 4

What are risk factors for pancreatic cancer?

Answer 4

Age>60
Smoking
Obesity/diet
Alcohol abuse
Pancreatitis 
Diabetes
Genetic predisposition (fan history and syndromes like FAP, Lynch, MEN)

Question 5

What are clinical features of pancreatic cancer?

Answer 5

Pain 
Obstructive jaundice
Weight loss
Anorexia
Migratory thrombophlebitis 

Often asymptomatic until invades adjacent structures

Question 6

What is precursor of invasive ductal adenocarcinoma?

Answer 6

Pancreatic intraepithelial neoplasia (PanIN)

Increases in prevalence with age

Question 7

Describe molecular progression of invasive ductal adenocarcinoma

Answer 7

Telomere shortening and KRAS mutation: PanIN1
Inactivation of CDKN2A: PanIN2
Inactivation of SMAD4: PanIN3
TP53: invasive ductal adenocarcinoma

Question 8

What is surgical procedure for tumors at head/neck of pancreas?

Answer 8

Whipple procedure (aka pancreaticoduodenectomy)

Question 9

Describe gross appearance of invasive ductal adenocarcinoma

Answer 9

Firm with poorly defined and infiltrative borders
Yellow-white in color
Hemorrhage/necrosis
Can appear cystic

Question 10

Describe histological appearance of ductal invasive adenocarcinoma

Answer 10

Desmoplastic stroma with fibroblasts and inflammatory cells
Can have haphazard irregularly shaped glands
Can have perineurial, vessel or lymphatic invasion
Irregular nuclear contours, N/C up, atypia

Question 11

What is the grading stages of adenocarcinoma?

Answer 11

Well differentiated==>poorly differentiated (how much of tumor is glandular)

Question 12

At what stage are most pancreatic cancers diagnosed?

Answer 12

Late stage with distant metastases

Question 13

What is 5 year survival for pancreatic ductal adenocarcinoma?

Answer 13

Real bad– 25% for local, 10% for regional 2% for distant

Question 14

How are differentiation and grade determined?

Answer 14

Differentiation= how close do neoplastic cells resemble non-neoplastic counterparts

Grade: look at mitoses and Ki-67 to measure aggressiveness

Question 15

Where does PanNET arise?

Answer 15

Usually a solitary, well-demarcated tumor that can occur anywhere along length of pancreas

Question 16

Describe gross appearance of well-differentiated PanNET

Answer 16

Well circumscribed tumor that is tan-pink and lacks necrosis

Question 17

Describe histology of well-differentiated PanNET

Answer 17

Well circumscribed lesion with surrounding capsule
Cords with dense collagenized stroma
Cells have abundant cytoplasm and salt/pepper chromatin

Question 18

What are two categories of NETs

Which ones are more common?

Answer 18

Function (hormone producing) and non-functioning

NF>insulinomas>gastrinomas>glucagonomas

Question 19

What are three major genes/pathways of well-differentiated PanNETs?

Answer 19

MEN1
PTEN/TSC2==>Activation of mTOR signaling
DAXX/ATRX

Question 20

Describe poorly differentiated PanNET: Prevalence, age, clinical behavior

Answer 20

2% of PanNETs
Occurs in adults
Highly aggressive clinical behavior– local and widespread distant mets

Question 21

Describe gross appearance of poorly differentiated PanNET

Answer 21

Infiltrative, aggressive

Question 22

Describe histological appearance of poorly-differentiated PanNET

Answer 22

small round blue cells with scant cytoplasm and hyper chromatic nuclei
Necrosis
Aggressive/infiltrative, metastatic

Question 23

What is a pancreatic pseudocyst?

How common is it?
When do you see it?

Answer 23

Cystic lesion that LACK true epithelial lining
75% of pancreatic cysts
Arises after bout of acute pancreatitis or traumatic injury

Question 24

What is serous cystadenoma?
Prevalence
Sex, age

Answer 24

Benign neoplasm that usually occurs in tail of pancreas
25% cystic neoplasms of pancreas
More common in women; 6th-7th decade

Question 25

How does pancreatic serous cyst adenoma present?

Answer 25

Nonspecific symptoms such as abdominal pain

Question 26

What is most common genetic abnormality of serous cyst adenoma?

Answer 26

Inactivation of VHL tumor suppressor gene

Question 27

Describe gross appearance of serous cystadenoma How is it normally treated?

Answer 27

Multi cystic with spongelike appearance Cured by surgery

Question 28

Mucinous cystic neoplasm: What is it? Location, sex, presentation

Answer 28

Painless growing mass in tail of pancreas almost always in women. It is filled with thick, tenacious mucin and is composed of columnar mutinous epithelium

Question 29

What are histological features of mucinous cystic neoplasm? (2)

Answer 29

Inner epithelial layer composed of tall mucin-secreting cells Dense cellular ovarian-type stroma

Question 30

What are possible progression of mucinous cystic neoplasm? (2)

Answer 30

Dysplasia | Invasive carcinoma

Question 31

Describe the epidemiology of intraductal papillary mucinous neoplasm (IPMN)

Answer 31

Most frequent cystic mucinous tumor in pancreas M>W; age=66yo Predominantly head of pancreas

Question 32

What are general features of IPMN?

Answer 32

Grossly visible intraductal proliferation of neoplastic mucin-producing cells Communicated with ductal system

Question 33

What are the distinctions between main duct vs. branch duct IOMN?

Answer 33

Main duct type: Diffusely or focally dilated, contains abundant mucus secretion Branch duct: More indolent course

Question 34

Describe gross features of IPMN

Answer 34

Branch: Circumscribed cystic lesion or cluster of mildly dilated ducts Main: Dilated duct

Question 35

Gallbladder adenocarcinoma Epidemiology: Prevalence, sex, geography, major risk factor

Answer 35

Most common malignancy of extra hepatic biliary tract W>M Chile/Bolivia/India Gallstones is major risk factor

Question 36

What is typical prognosis for gallbladder adenocarcinoma?

Answer 36

usually diagnosed at advanced stage-- 5 year survival

Question 37

Gross appearance of gallbladder adenocarcinoma

Answer 37

Large exophytic tumor | Calcification leading to porcelain gallbladder