Pancreatic Malignancies

Question 1

What are the two types of pancreatic cancer?

Answer 1

Ductal adenocarcinoma (85%)
Intraductal Pancreatic Mucinous Neoplasm (2-3%)

Question 2

What are some risk factors for the development of exocrine pancreatic cancer?

Answer 2

Cigarette smoking (1.5x increased relative risk)
Chronic pancreatitis (1.8% at 10 years, 4% at 20 years)

Question 3

What are some genetic changes that lead to the development of pancreatic cancer?

Answer 3

Mutations of K-RAS
Inactivation of p16
Inactivation of p53, SMAD4, BRCA2

Question 4

How do patients with pancreatic cancer typically present?

Answer 4

weakness, weight loss, anorexia, abdominal pain, jaundice, back pain

Question 5

What clinical symptoms are more common for cancers in the pancreatic head?

Answer 5

Painless jaundice
Steatorrhea
Weight loss

Question 6

How do you diagnosis pancreatic cancer?

Answer 6

Cholestatic liver pattern if biliary obstruction is present
Abdominal ultrasound for patients with jaundice
CT for patients with abdominal pain and weight loss (staging information too)

Question 7

Is carbonic anhydrase a helpful lab test for determining whether a patient has pancreatic cancer?

Answer 7

Not really – increased values may help you differentiation benign from cancer but values are often normal in early stages

Question 8

What is the treatment for pancreatic cancer?

Answer 8

Surgical resection – but 80-85% are unresectable at time of diagnosis because of distant metastases (liver) or invasion or encasement of the major blood vessels

Neoadjuvant therapy (before surgery) -- converts patient from nonresectable to resectable
Adjuvent therapy (after surgery ) -- patients with residual disease
Palliative -- surgical bypass for gastric outlet or biliary obstruction; stents: biliary, enteral

Question 9

What are some types of pancreatic cystic neoplasms?

Answer 9

Mucinous neoplasms = mucinous cystic neoplasma, Intraductal papillary mucinous neoplasm (IPMN)
Non-mucinous neoplasms = serous cystadenoma

Question 10

Who gets mucinous cystic neoplasms?

Answer 10

95% occur in women

Typically diagnosed > 40 yo

Question 11

What is a mucinous cystic neoplasm?

Answer 11

Ovarian-like storm that secretes mucin
Typically present in pancreatic body or tail
No communication with the pancreatic duct

Question 12

What are some symptoms of mucinous cystic neoplasm?

Answer 12

Usually asymptomatic
When symptomatic, can present with abdominal pain, recurrent pancreatitis, gastric outlet obstruction, palpable mass
Jaundice or weight loss more common with malignancy

Question 13

What is the treatment for mucinous cystic neoplasm?

Answer 13

Surgical resection regardless of size, d/t risk of malignancy

Question 14

What is an intraductal papillary mucinous neoplasm (IPMN)?

Answer 14

Mucin-producing papillary neoplasm of pancreatic duct

No predilection for location

Question 15

What are the different types of IPMN?

Answer 15

Main duct IPMN: involves the main pancreatic duct
Branch duct IPMN: involves the side branches of the main pancreatic duct
Mixed type IPMN: involves both the main pancreatic duct and its side branches

Question 16

What are some symptoms with IPMN?

Answer 16

Usually asymptomatic
Chronic pancreatitis d/t obstruction of pancreatic duct from mucous plugs
Back pain, jaundice, weight loss, anorexia, DM, anorexia concerning for malignancy

Question 17

What is the management for IPMN?

Answer 17

Main duct: surgical resection d/t risk of malignancy (70%)

Side branch: lower risk of malignancy, safe to monitor under 3cm, no pancreatitis

Question 18

What is a serous cyst adenoma?

Answer 18

25% of pancreatic cystic neoplasms 
Lined by glycogen-rich cells originating from pancreatic acinar cells 
Can arise anywhere in the pancreas
Usually in women over the age of 60 
Malignancy rare
*Central scar

Question 19

What are some symptoms of a serous cystadenoma?

Answer 19

Usually asymptomatic

Can present with abdominal pain, palpable mass, biliary obstruction, or gastric outlet obstruction when large

Question 20

How do you manage serous cystadenomas?

Answer 20

Conservative

Surgical resection if symptomatic

Question 21

What do you use to determine if a lesion is mucinous vs serous?

Answer 21

Endoscopic ultrasound/fine needle aspirate to sample cytology

Question 22

Name some examples of pancreatic neuroendocrine tumors.

Answer 22

Gastrinomas
Insulinomas
Somatostainomas
Glucagonomas
VIPomas

Question 23

How does insulinoma present?

Answer 23

Episodic hypoglycemia, most common pancreatic NET

Question 24

How does glucagonoma present?

Answer 24

Hyperglycemia, rash (necrolytic migratory erythema), chelitis, venous thrombosis

Question 25

How does a somatostatinoma present?

Answer 25

Diabetes mellitus (gastric inhibitory peptide), cholelithiasis (CCK), steatorrhea (secretin)

Question 26

How does VIPoma present?

Answer 26

Pancreatic cholera = watery diarrhea, hypokalemia, achlorhydria (WHDA)
VIP stimulates secretion of water into pancreatic juice and bile, inhibits gastric acid secretion

Question 27

What are some ways to diagnose NET?

Answer 27

Imaging: CT, MRI, Endoscopic ultrasound, somatostatin-receptor scintigraphy (high levels of somatostatin receptors)
Somatostatin analogues (octreotide) decrease secretion of a broad range of hormones
Proton-pump inhibitors for gastrinomas

Question 28

What is the treatment for NETs?

Answer 28

Surgical resection of primary tumor and/or metastases

*Metastatic disease present frequently for glucagonomas (50-100%), somatostatinomas (75%), and VIPomas (60-80%)

Question 29

What is the prognosis for NETs?

Answer 29

Well differentiated pancreatic NET are generally indolent

Poorly differentiated pancreatic NET have rapid progression