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- Carcinoma of the exocrine pancreas is the 4th most common cause of cancer death in the US.
- Rarely curable: Overall 1-year and 5-year relative survival rates of 25% and 5%, respectively.
- 60–70% occur in the head, 15% in the body, 5% in the tail; 20% diffusely involve the gland.
- <20% are localized at diagnosis. For localized, small cancers (<2 cm) with no lymph node metastases and no extension beyond the capsule, surgical resection has 5-year survival of 18–24%.
- In apparently resectable disease, 20–40% have unresectable lesions at surgery.
- Ampullary, duodenal, or distal bile duct tumors may mimic pancreatic carcinoma and are more likely to be resectable and curable.
- For advanced or unresectable cancers, survival is <1% at 5 years; most patients die within 1 year.
During 2003–2007, median age at diagnosis = 72 years; rare younger than age 45; after 45, occurrence rises sharply.Incidence
- An estimated 42,470 people diagnosed in 2009 (21,040 men and 21,420 women); 35,240 deaths
- More common in black and white races, 16.7 and 10.3 in 100,000 men and 14.4 and 10.3 in 100,000 women, respectively. Among Hispanic and Asian/Pacific Islanders there is an incidence of 10.9 and 8.3 in 100,000 men and 10.1 and 8.3 in 100,000 women, respectively (1).
In 2008, in the US, ~34,600 men and women (16,811 men and 17,846 women) were alive who had a history of pancreatic cancer.
- Smoking: Relative risk (RR) = 1.5; correlates with amount smoked
- Diabetes: RR = 2.1 (95% CI, 1.6–2.8); as many as 1 in 6 become diabetic within 6 months before diagnosis.
- Prior partial gastrectomy or cholecystectomy: 2–5-fold increased risk 15–20 years after gastrectomy
- Familial aggregation/genetic factors: 5–10% of patients have a 1st-degree relative with the disease, which confers a 9-fold increase in risk vs. the general population (2).
- Hereditary chronic pancreatitis: Cumulative risk by ages 50 and 75 is 10% and 54%, respectively.
- Chronic pancreatitis: Tropical and nontropical
- Non-O blood type
- High intake of dietary fat and obesity
- Alcohol: Recent data indicate a modest increase in risk confined to heavy alcohol consumers (3).
- Coffee intake, aspirin and NSAID use NOT regarded as a risk factors.
- Multifactorial: Mutational activation of oncogenes (e.g., K-ras mutation 90%); inactivation of tumor-suppressor genes (e.g., p16/CDKN2A [95%], TP53 [75–85%], SMAD4 [30%], and BRCA2 genes [10%]); and defects in DNA mismatch repair genes (e.g., hMLH1 and hMSH2 [4% of tumors])
- Hereditary pancreatitis: Cumulative risk of pancreatic cancer in affected family members by age 70 is estimated to be 40%.
- Inherited cancer syndromes:
- Peutz-Jeghers syndrome: Related genes: PRSS1 and STK 11; lifetime risk is as high as 36%.
- Hereditary breast/ovarian cancer: 5% lifetime risk for pancreas cancer; related genes: BRCA2 and BRCA1
- Familial atypical multiple-mole melanoma syndrome: 19% lifetime risk; related gene: CDKN2A
- Ataxia-telangiectasia: Related gene: ATM
- Li-Fraumeni syndrome: Related gene: p53
Routine screening is not recommended. Even with a strong family history or predisposition syndromes, utility and cost-effectiveness of screening are unclear.
- Pancreatic intraepithelial neoplasia: Best characterized precursor lesion. Progression to severe dysplasia is marked by expression of multiple oncogenes.
- K-ras mutations and telomere shortening are the earliest known genetic abnormalities, even in low-grade intraepithelial neoplasia.
Commonly Associated Conditions
- See “Genetics.”
- Chronic pancreatitis; diabetes mellitus; inherited cancer syndromes, as noted above