Pancreatic Malignancies

Question 1

What is the racial and sex makeup of pancreatic exocrine cancer?
What is the primary type of histology?
What is the survival rate without surgery?

Answer 1

Male, African-American
Ductal adenocarcinoma (85%)
5% five-year survival without surgery

Question 2

What are the options for exocrine pancreatic cancer therapy?

Answer 2

Curative surgery (resection)
80-85% are unresectable at time of diagnosis
Metastatses: liver or invasion/encasement of the major blood vessels
Whipple procedure – Pancreatic head lesions

Neoadjuvant therapy (pre-surgery)
Convert patient from nonresectable to resectable

Adjuvant therapy (after surgery)
Patients with residual disease

Palliative
Surgical bypass for gastric outlet or biliary obstruction
Stents: Biliary, enteral

Question 3

What are the risk factors for exocrine pancreatic cancer?

Answer 3

Cigarette smoking: 1.5 times relative risk
Chronic pancreatitis: More with longer duration

Question 4

What is the pathogeneis of pancreatic exocrine cancer?

Answer 4

Stepwise process
Oncogene activation: k-Ras
Inactivation of p16, p53, SMAD4, BRCA2

Question 5

What is the presentation of the exocrine pancreatic cancers?

Answer 5

Weakness, weight loss, anorexia, abdominal pain, jaundice (50%), back pain (50%)
Painless jaundice, steatorrhea, weight loss – Pancreatic head

Question 6

What are the diagnostic tools for pancreatic malignancies?

Answer 6

Cholestatic liver pattern if biliary obstruction is present

Abdominal US for patient with jaundice

CT for patients with ab pain and weight loss

Carbonic Anhydrase 19-9
Often normal in early stage so not useful for screening
Increased values may help differentiate benign disease from cancer

Question 7

What is the prognosis of pancreatic exocrine tumors?

Answer 7

Stage I 5 year: 31.4%
Stage IV 5 year: 2.8%
Worsens with staging and bad in general

Question 8

What are the types of pancreatic cystic neoplasms?

Answer 8

Mucinous neoplasms:
Mucinous cystic neoplasm
Intraductal papillary mucinous neoplasm

Non-mucinous neoplasm:
Serious cystadenoma

Question 9

What is the age and sex bias of mucinous cystic neoplasm?
What is a mucinous cystic neoplasm?
Where is it typically located?

Answer 9

95% occur in women
Typically diagnosed > age 40

Ovarian-like stroma that secretes mucin

Typically in the pancreatic body or tail
No communication with the pancreatic duct

Question 10

What are the symptoms for mucinous cystic neoplams?
What is the treatment?

Answer 10

Usually asymptomatic
Ab pain, recurrent pancreatitis, gastric outlet obstruction, palpable mass
Jaundice and/or weight loss more common with malignancy

Surgical resection regardless of size due to malignancy

Question 11

What is an IPMN?
What is the sex/age distribution?
Where is it located/what are the types?

Answer 11

Mucin-producing papillary neoplasms of the pancreatic duct

Equal sex distribution
Incidence peak over age 50

No specific predilection for location

Types
Main duct: 70%
Branch duct: 20%
Mixed: 10%

Question 12

What is the appearance of IPMN on CT and ERCP?

What are the symptoms?

Answer 12

Thick mucus visible on CT, called fisheye on ERCP

Typically asymptomatic
Chronic pancreatitis due to obstruction
Back pain, jaundice, weight loss, anorexia, DM all signs of malignancy

Question 13

What is the management of IPMN?

Answer 13

Main duct: Surgical resection due to risk of malignancy
Side branch: Lower risk of malignancy
Safe to monitor under select circumstances (cyst size < 3 cm, no pancreatitis)

Question 14

What percentage of pancreatic cystic neoplasms are serous cystadenoma?
What is it lined by?
Where does it arise?
What is the age/sex bias?
How common is malignant degeneration?

Answer 14

25% of pancreatic cystic neoplasms
Lined by glycogen-rich cells originating from pancreatic acinar cells
Can arise anywhere in the pancreas
Usually diagnosed in women over the age of 60
Malignant degeneration very rare

Question 15

What are the symptoms of serous cystadenoma?
What is the management?

Answer 15

Symptoms

Usually asymptomatic
Same symptoms of obstruction if it gets large

Management

Conservative
Only remove if too long

Question 16

How is mucinous distinguished versus serous cytic lesions?

Answer 16

Endoscopic ultrasound
Fine needle aspirate

Question 17

What is the purpose for fine needle aspirate?

Answer 17

Sampling using thin needle (19-22 gauge)
Cytology: sample of cyst wall
Cystic fluid sampling
Confocal microscopy

Question 18

What are the types of pancreatic neuroendocrine tumors?
What do they present as?

Answer 18

Gastrinomas: ZE syndrome
Insulinomas: Episodic hypoglycemia, most common pancreatic NET

Somatostatinomas – All effects due to inhibition of various enzymes: DM (gastric inhibitor peptide), Cholelithiasis (CCK), Steatorrhea (Secretin)

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

VIPomas (Pancreatic cholera): Watery diarrhea, hypokalema, achlorhydria (WDHA)
Stimulate secretion of water into pancreatic juice and bile, inhibits gastric acid secretion

Question 19

What is the epidemiology of pancreatic NET?

Answer 19

Together 1 in 100,000
Typically diagnosed from ages 40-60
Mostly sporadic, but can be associated with inherited syndromes (MEN-1)

Question 20

What is imaging used for NETs?
What drug is used as a somatostatin analogue?

Answer 20

CT

MRI

Endoscopic US

Somatostatin-receptor scintigraphy
Most NETs (not insulinomas) have high levels of somatostatin receptors
Octreorides can be used to identify receptors

Question 21

What are the treatment options of NETs?

Answer 21

Octreoride decreases secretion from many of these (not insulinomas)
PPIs for gastrinomas
Surgical resection is the only real treatment
Liver resection if metastases

Question 22

What is the prognosis of NET?

Answer 22

Metatstatic disease present frequently for glucagonomas (50-100%), somatostatinomas (75%) and VIPomas (60-80%)
Relatively slow growing metastases (“good”)
Well differentiated generally indolent
Poorly differentiated NET: rapid progression