Screening and Prevention Recommendations for Individuals with Family History of Pancreatic Cancer
Individuals with a family history of pancreatic cancer should undergo surveillance with MRI/MRCP and EUS starting at age 50 or 10 years younger than the earliest pancreatic cancer diagnosis in the family, whichever comes first. 1
Who Should Be Screened
Pancreatic cancer screening is recommended for the following high-risk individuals:
Genetic Mutation Carriers:
- All patients with Peutz-Jeghers syndrome (STK11/LKB1 mutation) - regardless of family history 1
- All CDKN2A mutation carriers - regardless of family history 1
- Carriers of mutations in the following genes WITH at least one affected first-degree relative:
- BRCA2
- BRCA1
- PALB2
- ATM
- MLH1/MSH2/MSH6 (Lynch syndrome) 1
Family History Criteria (without known genetic mutations):
- Individuals with ≥3 affected relatives on the same side of the family, with at least one being a first-degree relative
- Individuals with ≥2 affected first-degree relatives
- Individuals with ≥2 affected relatives on the same side of the family, with at least one being a first-degree relative 1
When to Begin Screening
The timing of screening initiation depends on genetic status and family history:
- Familial pancreatic cancer kindreds (no known mutation): Age 50 or 10 years younger than the youngest affected relative 1, 2
- Specific mutation carriers:
- Hereditary pancreatitis: Age 40 or 20 years after first pancreatitis attack 1
- New-onset diabetes in a high-risk individual should prompt immediate screening regardless of age 1, 2
How to Screen
The recommended screening protocol includes:
Baseline Screening:
Follow-up Screening:
- Alternate MRI/MRCP and EUS 1
- Regular testing of fasting blood glucose and/or HbA1c 1
- CA19-9 for individuals with concerning features on imaging 1
Additional Testing Based on Findings:
- EUS-FNA for:
- Solid lesions ≥5mm
- Cystic lesions with worrisome features
- Asymptomatic main pancreatic duct strictures 1
- CT for:
- Solid lesions
- Asymptomatic main pancreatic duct strictures without mass 1
Screening Intervals
- 12 months: For individuals with no abnormalities or only non-concerning abnormalities (e.g., pancreatic cysts without worrisome features) 1, 2
- 3-6 months: For concerning abnormalities that don't immediately warrant surgery 1, 2
- Immediate evaluation: For new-onset diabetes in high-risk individuals 1
Genetic Testing Considerations
Genetic testing and counseling should be considered for individuals with:
- Family history meeting criteria for familial pancreatic cancer
- Personal or family history of pancreatic cancer and Ashkenazi Jewish ancestry (higher prevalence of BRCA1/2 mutations) 1, 2
Goals of Surveillance
The primary goals of pancreatic cancer surveillance are to detect and treat:
- Stage I pancreatic cancer confined to the pancreas with negative margins
- Pancreatic cancer precursor lesions with high-grade dysplasia (PanIN or IPMN) 1
Important Caveats
- Screening should be performed at high-volume specialty centers with expertise in pancreatic diseases 1, 2
- Participation in a registry or research protocol is recommended when possible 2
- Screening is not recommended for average-risk individuals 2
- Consider discontinuing screening when patients are more likely to die from non-pancreatic cancer causes or are not surgical candidates 2
- The limitations and potential risks of pancreatic cancer screening should be discussed with patients before initiating surveillance 2
Surgical Management
When concerning lesions are detected, surgical resection should be performed at high-volume centers by experienced pancreatic surgeons 1, 2. An oncological radical resection is indicated for suspected pancreatic cancer, while partial pancreatectomy may be suitable for small suspected pancreatic cancers (≤1cm) 1.