Pancreatic Cancer Screening in a Population at High Risk

Pancreatic cancer is one of the diseases with the worst prognosis, which is mainly due to the initial asymptomatic prognosis. Unfortunately, the incidence of this disease in the Czech Republic is still increasing. In a certain proportion of patients, it is possible to predict the disease, e.g. due to family burdens. Regular follow-up of such individuals is the subject of the SCREPAN study: "Pancreatic Cancer Screening in High-Risk Persons".

Sex

All

Minimum age

18 Years

Inclusion Criteria:

* willing to participate in the study
* age 18+
* arms specific criteria:

A:

* chronic pancreatic disease in the context of cystic fibrosis or chronic pancreatitis
* age 50+

B1:

* confirmed Peutz-Jegherson syndrome (mutSTK11) + age over 35 years or 10 years earlier than pancreatic ductal adenocarcinoma was diagnosed in the youngest family member
* familial melanoma syndrome (mutCDKN2A) + age over 40 years or 10 years before pancreatic ductal adenocarcinoma was diagnosed in the youngest family member
* confirmed hereditary pancreatitis (mutPRSS1) + age over 40 years or 20 years after the first attack

B2:

* confirmed diagnosis of hereditary syndrome (Lynch syndrome /mutMLH1, mutMSH2, mutMSH6, mutPMS2, mutEPCAM/, HBOC /mutBRCA1, mutBRCA2, mutPALB2, mutATM/, familial adenomatous polyposis /mutAPC/, Li-Fraumeni syndrome /mutTP53/)
* at least one relative with a diagnosis of pancreatic ductal adenocarcinoma in family anamnesis at the same time (Grade I or II relative)
* age over 50 years, or 10 years before the pancreatic ductal adenocarcinoma was diagnosed in the youngest relative - which comes first

C:

* positive family anamnesis of pancreatic ductal adenocarcinoma without hereditary syndrome context
* age 50+ or 10 years earlier than the youngest relative with pancreatic ductal adenocarcinoma - screening is recommended for all first-degree relatives of affected family members

Exclusion Criteria:

* Inability to undergo radical curative surgery for a pancreatic tumor.
* Inability to undergo scheduled imaging examinations.
* Incurable malignant cancer.

• BACKGROUNDCanto 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; International Cancer of Pancreas Screening (CAPS) Consortium. International Cancer of the Pancreas Screening (CAPS) Consortium summit on the management of patients with increased risk for familial pancreatic cancer. Gut. 2013 Mar;62(3):339-47. doi: 10.1136/gutjnl-2012-303108. Epub 2012 Nov 7. PMID 23135763
• BACKGROUNDSyngal S, Brand RE, Church JM, Giardiello FM, Hampel HL, Burt RW; American College of Gastroenterology. ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62; quiz 263. doi: 10.1038/ajg.2014.435. Epub 2015 Feb 3. PMID 25645574
• BACKGROUNDStoffel EM, McKernin SE, Brand R, Canto M, Goggins M, Moravek C, Nagarajan A, Petersen GM, Simeone DM, Yurgelun M, Khorana AA. Evaluating Susceptibility to Pancreatic Cancer: ASCO Provisional Clinical Opinion. J Clin Oncol. 2019 Jan 10;37(2):153-164. doi: 10.1200/JCO.18.01489. Epub 2018 Nov 20. PMID 30457921
• BACKGROUNDAbe T, Blackford AL, Tamura K, Ford M, McCormick P, Chuidian M, Almario JA, Borges M, Lennon AM, Shin EJ, Klein AP, Hruban RH, Canto MI, Goggins M. Deleterious Germline Mutations Are a Risk Factor for Neoplastic Progression Among High-Risk Individuals Undergoing Pancreatic Surveillance. J Clin Oncol. 2019 May 1;37(13):1070-1080. doi: 10.1200/JCO.18.01512. Epub 2019 Mar 18. PMID 30883245
• BACKGROUNDBartsch DK, Slater EP, Carrato A, Ibrahim IS, Guillen-Ponce C, Vasen HF, Matthai E, Earl J, Jendryschek FS, Figiel J, Steinkamp M, Ramaswamy A, Vazquez-Sequeiros E, Munoz-Beltran M, Montans J, Mocci E, Bonsing BA, Wasser M, Kloppel G, Langer P, Fendrich V, Gress TM. Refinement of screening for familial pancreatic cancer. Gut. 2016 Aug;65(8):1314-21. doi: 10.1136/gutjnl-2015-311098. Epub 2016 May 24. PMID 27222532
• BACKGROUNDHarinck F, Konings IC, Kluijt I, Poley JW, van Hooft JE, van Dullemen HM, Nio CY, Krak NC, Hermans JJ, Aalfs CM, Wagner A, Sijmons RH, Biermann K, van Eijck CH, Gouma DJ, Dijkgraaf MG, Fockens P, Bruno MJ; Dutch research group on pancreatic cancer surveillance in high-risk individuals. A multicentre comparative prospective blinded analysis of EUS and MRI for screening of pancreatic cancer in high-risk individuals. Gut. 2016 Sep;65(9):1505-13. doi: 10.1136/gutjnl-2014-308008. Epub 2015 May 18.