Friday, November 9, 2012

Pancreatic cancer screening in PJS

This is important because pancreatic cancer screening guidelines for PJS people vary dramatically by expert, center and country. The experts agree we need screening, but don't agree on what type, age to begin or frequency of screening. 

Still, it's an important start.

International Cancer of the Pancreas Screening (CAPS) Consortium
summit on the management of patients with increased risk for familial pancreatic
cancer. Gut. 2012 Nov 7.

Canto MI, Harinck F, Hruban RH, Offerhaus GJ, Poley JW, Kamel I, Nio Y,
Schulick RS, Bassi C, Kluijt I, Levy MJ, Chak A, Fockens P, Goggins M, Bruno M;
on behalf of the International Cancer of the Pancreas Screening (CAPS)

PubMed PMID: 23135763.

Who should be screened?
Since the incidence of PC in the general population is low (lifetime risk 1.3%), screening is not recommended for the general population, but instead for individuals considered to be at high risk of developing the disease (ie, >5% lifetime risk, or fivefold increased RR). The main tool used to quantify PC risk is still the family history; risk stratification is determined from the number of affected family members and the relationships among at-risk individuals.15 Gene testing can identify a family's underlying genetic susceptibility, but it has a limited role because the genetic basis of much of the inherited susceptibility to PC remains unexplained. Additional PC susceptibility genes may be discovered in the near future that should improve our ability to identify individuals who would benefit most from pancreatic screening.


Patients with Peutz–Jeghers syndrome, regardless of family history, should be considered for screening (agree 96%, grade moderate, ‘do it’).

Patients with Peutz–Jeghers syndrome (who generally carry germline STK11 gene mutations) have a very high (132-fold) risk of PC. Lifetime cumulative risk to age 65–70 for PC in patients with Peutz–Jeghers syndrome is 11–36%.

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