Pancreatic Cancer

Question 1

What kinds of tumors are normally called “pancreatic cancers”?

Answer 1

pancreatic ductal adenocarcinoma (>90%)
endocrine tumors
lymphomas
cystic neoplasms
squamous cell carcinoma
giant cell
solid pseudopapillary neoplasms
others

Question 2

List the risk factors for pancreatic cancer?

Answer 2

current cigarette use (1.7-2.2)
current pipe or cigar use (1.5)
>3 alcoholic drinks per day (1.2-1.4)
chronic pancreatitis (13.3)
BMI > 40 kg/m2, male (1.5)
BMI > 40 kg/m2, female (2.8)
DM, type I (2.0)
DM, type 2 (1.8)
cholecystectomy (1.2)
gastrectomy (1.5)
H. pylori infection (1.4)

Question 3

What is Pancreatic Intraepithelial Neoplasm (PanIN)?

Answer 3

small intraductal lesions formed by abnormal proliferation of ducts
PanIN demonstrates varying degrees of dysplasia (PanIN-1, PanIN-2, and PanIN-3)
some pancreatic cancers arise from PanIN, but not all PanIN becomes cancers
unable to visualize clearly on imaging

Question 4

What are Mucinous Cystic Neoplasms?

Answer 4

ovarian stroma, possibly arising from ovarian rests within pancreas
invasive carcinoma 6-36%

Question 5

What is Intraductal papillary Mucinous Neoplasm?

Answer 5

branch duct (~25% risk of malignancy)
main duct (~70% risk of malignancy)

Question 6

What clinical symptoms are associated with pancreatic cancer?

Answer 6

abdominal pain
obstructive jaundice (head)
weight loss
nausea and vomiting
anorexia
malaise
Courvoisier's sign (palpably distended non-tender gallbladder)
Trousseau's sign (thrombosis)

Question 7

How is Pancreatic cancer diagnosed?

Answer 7

CT scan

endoscopic US

Question 8

How is Pancreatic cancer treated?

Answer 8

surgery (pancreaticoduodenectomy/Whipple, pancreatectomy)

Chemotherapy (Gemcitabine, FOLFIRINOX

Question 9

What is the incidence of pancreatic cancer in patients with affected fist degree relatives?

Answer 9

10-15% of patients with pancreatic cancer have a familial aggregation or an inherited predisposition
1 FDR- 41 per 100,000
2 FDR- 58 per 100,000
3+ FDR- 288 per 100,000

Question 10

What inherited syndromes predispose to pancreatic cancer?

Answer 10

HBOC
Ataxia Telangiectasia
Familial Atypical Multiple Mole Melanoma (FAMMM)
Peutz-Jeghers
Lynch Syndrome
Hereditary pancreatitis
Familial Polyposis

Question 11

What Pancreatic screening options are available?

Answer 11

Endoscopic Ultrasound (EUS)- requires sedation, invasive procedure, ability to biopsy abnormalities
Magnetic Resonance Imaging (MRI)- non-invasive, unable to biopsy, patient tolerance

Question 12

When should genetic counseling and germline testing for pancreatic cancer?

Answer 12

all individuals with exocrine pancreatic cancer

first-degree relatives of individuals diagnosed with exocrine pancreatic cancer

Question 13

What genes are associated with pancreatic cancer?

Answer 13

ATM
BRCA1
BRCA2
CDKN2A
MLH1
MSH2
MSH6
EPCAM
PALB2
STK11
TP53