# Familial pancreatic cancer and inherited risk (who qualifies for surveillance)

Source: https://onco.cc/terms/familial-pancreatic-cancer/  
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## TL;DR

Familial pancreatic cancer means at least two close relatives on the same side of the family have had the disease without a known gene fault to explain it. Members of such families have several times the usual risk, rising steeply with the number of relatives affected. With carriers of certain gene faults, they are the people offered yearly MRI or endoscopic ultrasound in surveillance programmes.

## Summary

Definition and risk. A familial pancreatic cancer kindred has at least one pair of first-degree relatives with pancreatic cancer. In the Johns Hopkins National Familial Pancreas Tumor Registry (5,179 people from 838 kindreds), members of such kindreds with a first-degree relative affected had 9.0 times the expected incidence, rising to 6.4 times with two and 32 times with three affected first-degree relatives, while members of sporadic kindreds (1.8 times) and genetically unrelated spouses had no significant excess (Klein 2004); Cancer Research UK puts the risk from one first-degree relative at 62 to 76 percent higher. Genes explain a minority: 5.5 percent of 3,030 unselected patients carried a pathogenic variant in ATM, BRCA2, BRCA1, CDKN2A, TP53 or MLH1 (7.9 percent of those with a family history, 5.2 percent without) (Hu 2018), and most carriers have no family history (Shindo 2017); the syndromes with the highest relative risks are Peutz-Jeghers (more than 100 times), familial atypical multiple mole melanoma with CDKN2A (13 to 38 times) and hereditary pancreatitis with PRSS1 (more than 50 times) (Cancer Research UK). Who is offered surveillance. NICE NG85 offers it to hereditary pancreatitis with PRSS1, to BRCA1, BRCA2, PALB2 or CDKN2A carriers with one or more affected first-degree relatives, and to Peutz-Jeghers syndrome (1.1.15), and asks clinicians to consider it for two or more affected first-degree relatives across two generations and for Lynch syndrome with an affected first-degree relative (1.1.16), by MRI/MRCP or EUS (1.1.17). The International CAPS consortium (2020) starts familial surveillance at 50 or 55, or ten years before the youngest affected relative, uses EUS and MRI/MRCP yearly and added ATM carriers with one affected relative (Goggins 2020). What surveillance finds. In CAPS5, 77.8 percent of surveillance-detected cancers were stage I, and across CAPS1 to 5 five-year survival for screen-detected disease was 73.3 percent (Dbouk 2022); the Dutch programme found a 9.3 percent cumulative incidence in mutation carriers but none in mutation-negative familial kindreds over 63 months (Overbeek 2022); the Dutch CDKN2A cohort had a 20.7 percent cumulative incidence by age 70 with 83.3 percent of cancers resectable at imaging (Klatte 2022); the UK EUROPAC registry found one cancer and 41 cystic lesions in 321 people, the branch-duct IPMNs unrelated to inherited risk (Sheel 2019). The PRECEDE consortium is enrolling 20,000 people to standardise the approach (Gonda 2021; its trial record is linked). Practical route in the UK: ask the oncology team or GP for a clinical genetics referral; germline testing is offered to every patient with pancreatic cancer, and a positive result opens surveillance to relatives.

## Fields

- Kind: Term
- Last checked: 2026-09-24
- Also known as: Familial pancreatic cancer kindred; FPC; Hereditary pancreatic cancer; Inherited pancreatic cancer risk; Pancreatic cancer in the family; Two first-degree relatives with pancreatic cancer; High-risk individual for pancreatic cancer; High-risk individual; HRI; High-risk individuals in pancreatic surveillance
- Tags: gi; pancreatic

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Pancreatic_cancer
- Klein, Cancer Res 2004: prospective risk of pancreatic cancer in familial pancreatic cancer kindreds: https://doi.org/10.1158/0008-5472.can-03-3823
- Hu, JAMA 2018: inherited germline mutations in cancer predisposition genes and pancreatic cancer risk (3,030 patients): https://doi.org/10.1001/jama.2018.6228
- Shindo, J Clin Oncol 2017: deleterious germline mutations in apparently sporadic pancreatic adenocarcinoma: https://doi.org/10.1200/jco.2017.72.3502
- NICE NG85: pancreatic cancer in adults (diagnosis 1.1, staging 1.3, nutrition 1.6, biliary obstruction 1.7, resectable disease 1.8): https://www.nice.org.uk/guidance/ng85/chapter/Recommendations
- Goggins, Gut 2020: CAPS consortium updated recommendations for individuals at increased risk of familial pancreatic cancer: https://doi.org/10.1136/gutjnl-2019-319352
- Dbouk, J Clin Oncol 2022: the multicenter CAPS5 study, impact of surveillance on stage and survival: https://doi.org/10.1200/jco.22.00298
- Overbeek, Gut 2022: long-term yield of pancreatic cancer surveillance in 366 Dutch high-risk individuals: https://doi.org/10.1136/gutjnl-2020-323611
- Klatte, J Clin Oncol 2022: 20 years of surveillance in 347 germline CDKN2A carriers: https://doi.org/10.1200/jco.22.00194
- Sheel, Am J Gastroenterol 2019: cystic lesions found by EUROPAC secondary screening of familial pancreatic cancer kindreds: https://doi.org/10.1038/s41395-018-0395-y
- Gonda, Gastroenterology 2021: PRECEDE consortium recommendations for a more organised approach to early detection: https://doi.org/10.1053/j.gastro.2021.08.036
- CRUK: pancreatic cancer risk factors (professional): https://www.cancerresearchuk.org/health-professional/cancer-statistics/statistics-by-cancer-type/pancreatic-cancer/risk-factors
- CRUK: screening for pancreatic cancer (no national programme; high-risk surveillance): https://www.cancerresearchuk.org/about-cancer/pancreatic-cancer/getting-diagnosed/screening

## Connected records

- terms: [CAPS: the Cancer of the Pancreas Screening consortium and its surveillance studies (CAPS1 to CAPS5)](https://onco.cc/terms/caps-consortium-pancreatic-screening/), [Germline BRCA mutation (gBRCA)](https://onco.cc/terms/gbrca-mutation/), [Germline vs somatic mutations](https://onco.cc/terms/germline-vs-somatic/), [High-risk stigmata and worrisome features of pancreatic cysts (IPMN and MCN surgical criteria)](https://onco.cc/terms/pancreatic-cyst-high-risk-stigmata/), [Lynch syndrome](https://onco.cc/terms/lynch-syndrome/), [Pancreatic intraepithelial neoplasia (PanIN), the microscopic precursor of pancreatic cancer](https://onco.cc/terms/panin/), [Stage shift](https://onco.cc/terms/stage-shift/)
- cancers: [BRCA or PALB2-mutant pancreatic ductal adenocarcinoma](https://onco.cc/cancers/brca-palb2-pdac/), [Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors](https://onco.cc/cancers/ipmn-cystic-precursors/), [Pancreatic ductal adenocarcinoma](https://onco.cc/cancers/pancreatic/)
- technologies: [Endoscopic ultrasound and EBUS systems](https://onco.cc/technologies/endoscopic-ultrasound-systems/), [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/), [High-risk pancreatic surveillance (CAPS / PRECEDE)](https://onco.cc/technologies/pancreatic-surveillance/), [MRI](https://onco.cc/technologies/mri/)
- targets: [ATM](https://onco.cc/targets/atm/), [BRCA1 / BRCA2 (HRD)](https://onco.cc/targets/brca/), [CDKN2A](https://onco.cc/targets/cdkn2a/), [PALB2](https://onco.cc/targets/palb2/), [STK11](https://onco.cc/targets/stk11/)
- institutions: [Pancreatic Cancer UK](https://onco.cc/institutions/pancreatic-cancer-uk/)
- trials: [EUROPAC](https://onco.cc/trials/europac/), [PRECEDE](https://onco.cc/trials/precede/)

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