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. Author manuscript; available in PMC: 2022 Aug 9.
Published in final edited form as: JAMA. 2021 Sep 7;326(9):851–862. doi: 10.1001/jama.2021.13027
General Facts
Approximately 60 000 diagnoses per year in the US
Incidence of about 1% over lifetime
The tenth to eleventh leading cause of cancer in the US
Third leading cause of cancer-related mortality
5-year survival (all-comers), 10%
Median age at diagnosis, 71 years
Male/female incidence ratio: 1.3/1.0
50% of patients present with metastatic disease (AJCC stage IV)
30% of patients present with locally advanced disease (AJCC stage III)
20% of patients present with localized resectable disease (AJCC stage I and II)
Most common causative germline alterations: BRCA2, BRCA1, ATM, PALB2
Common sites of metastasis: liver, lymph node, lung, and peritoneum
Rare sites of metastasis: skin, brain, and leptomeninges
Lifestyle Risk Factors
Tobacco
Excess alcohol consumption (chronic pancreatitis)
Obesity (body mass index >30), metabolic disorders, low levels of physical activity
Diet: high fat, polyunsaturated fats, processed meats
Genetic Risk Factors a
Hereditary breast and ovary cancer syndrome (BRCA1/2, PALB2; 5%–9%)
Ataxia-telangiectasia (ATM; approximately 3%–4%)
Familial atypical multiple mole and melanoma syndrome (CDKN2A, p16; <1%)
Lynch syndrome (MLH1, MSH2, MSH6, PMS2, EPCAM; <1%)
Hereditary pancreatitis (PRSS1, SPINK1; <1%)
Peutz-Jeghers syndrome (STK11; <1%)

Abbreviation: AJCC, American Joint Committee on Cancer.

a

Percentages indicate the frequency per 100 unselected patients diagnosed with pancreas cancer.