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International Cancer of the Pancreas Screening (CAPS) Consortium consensus on screening for pancreatic cancer in patients with increased risk for familial pancreatic cancer

International Cancer of the Pancreas Screening (CAPS) Consortium consensus on screening for pancreatic cancer in patients with increased risk for familial pancreatic cancer
Who?
  • All patients with Peutz-Jeghers syndrome (carriers of a germline LKB1/STK11 gene mutation)
  • All carriers of a germline CDKN2A mutation
  • Carriers of a germline BRCA2, BRCA1, PALB2, ATM, MLH1, MSH2, or MSH6 gene mutation with at least 1 affected first-degree blood relative
  • Individuals who have at least 1 first-degree relative with pancreatic cancer who in turn also has a first-degree relative with pancreatic cancer (familial pancreatic cancer kindred)
When (at what age)?
  • Age to initiate surveillance depends on an individual's gene mutation status and family history
  • Familial pancreatic cancer kindred (without a known germline mutation):
    • Start at age 50 or 55*, or 10 years younger than the youngest affected blood relative
  • Mutation carriers:
    • For CDKN2A and Peutz-Jeghers syndrome, start at age 40
    • For BRCA2, ATM, PALB2, BRCA1, and MLH1/MSH2, start at age 45 or 50, or 10 years younger than the youngest affected blood relative
  • There is no consensus on the age to end surveillance
How?
At baseline
  • MRI/MRCP + EUS + fasting blood glucose and/or HbA1c
During follow-up
  • Alternate MRI/MRCP and EUS (no consensus on if and how to alternate)
  • Routinely test fasting blood glucose and/or HbA1c
On indication
  • Serum CA 19-9:
    • If concerning features on imaging
  • EUS-FNA only for:
    • Solid lesions of ≥5 mm
    • Cystic lesions with worrisome features
    • Asymptomatic MPD strictures (with or without mass)
  • CT only for:
    • Solid lesions, regardless of size
    • Asymptomatic MPD strictures of unknown etiology (without mass)
Intervals and surgery
  • 12 months:
    • If no abnormalities or only nonconcerning abnormalities (eg, pancreatic cysts without worrisome features)
  • 3 or 6 months:
    • If concerning abnormalities for which immediate surgery is not indicated (refer to UpToDate text)
  • Surgery:
    • If positive FNA and/or a high suspicion of malignancy on imaging (refer to UpToDate text)
  • When surgery is indicated, perform an oncologic radical resection at a specialty center
Goals
  • The goal of surveillance is to detect and treat the following pathologic lesions:
    • Stage I pancreatic cancer, confined to the pancreas, resected with negative margins
    • Pancreatic cancer precursor lesions with high-grade dysplasia (PanIN or IPMN)
LKB1: liver kinase B1; STK11: serine/threonine kinase 11; CDKN2A: cyclin-dependent kinase inhibitor 2A; BRCA: breast cancer associated; PALB2: partner and localizer of BRCA2; ATM: ataxia telangiectasia mutated; MLH1: mutL homolog 1; MSH: mutS homolog; MRI/MRCP: magnetic resonance imaging/magnetic retrograde cholangiopancreatography; EUS: endoscopic ultrasound; HbA1c: hemoglobin A1c; CA 19-9: carbohydrate antigen 19-9; FNA: fine-needle aspiration; MPD: main pancreatic duct; CT: computed tomography; PanIN: pancreatic intraepithelial neoplasia; IPMN: intraductal papillary mucinous neoplasm.
* Consensus as to when to start surveillance was not reached.
¶ Literature-based recommendation.
Reproduced with permission from: Goggins M, Overbeek KA, Brand R, et al. Management of patients with increased risk for familial pancreatic cancer: updated recommendations from the International Cancer of the Pancreas Screening (CAPS) Consortium. Gut 2020; 69(1):7-17. Copyright © 2020 BMJ Publishing Group Ltd.
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