Overview
IRFARPC is a multicenter national registry designed to study the diagnosis and predisposing factors of subjects with an inherited increased risk for pancreatic cancer.
Description
Pancreatic cancer is a devastating disease with a dismal prognosis. One of the ways to improve survival might be early detection.
Within years, many predisposing diseases or genetic conditions have been identified, thus screening/surveillance have been established worldwide.
A registry of subjects at risk of pancreatic cancer will has been built up to investigate the possibility of diagnosis pancreatic cancer, or one if its predisposing lesions.
Inclusion criteria will be adopted as "enrollment criteria". According to this, individuals > 18 years old will be enrolled; their familiar history and/or genetic predisposition will be collected, as well as current or previous medical records/medications data. Thereafter, according to specific age-based criteria, those individuals initially enrolled will be will be considered for a "surveillance protocol" ("interventional follow-up criteria") and they will be submitted to Cholangio-Wirsung Magnetic Resonance or Pancreatic Endoultrasonography according to the pancreatologist's prescription at each participating center.
Individuals suffering from the following conditions will be enrolled:
familial pancreatic cancer; Peutz-Jeghers syndrome; a known BRCA-2, BRCA-1, PALB2, or p16 mutation with at least one first- or second-degree relative suffering from pancreatic cancer; hereditary pancreatitis; FAMMM syndrome; Lynch syndrome with at least one first- or second-degree relative suffering from pancreatic cancer.
Eligibility
Inclusion Criteria to enter the registry:
- individuals with at least two relatives suffering from pancreatic cancer, with at least 1 first-degree and until the third-degree
- subjects with known genetic mutation of BRCA2, BRCA1, p16, PALB2 with at least 1 first- or 2nd-degree relative suffering from pancreatic cancer
- subjects suffering from FAMMM Syndrome
- subjects suffering from Peutz-Jeghers Syndrome
- subjects suffering from PRSS-1- or CFTR- or SPINK-1- related pancreatitis
- subjects suffering from Lynch syndrome with at least 1 first- or 2nd-degree relative suffering from pancreatic cancer
Inclusion criteria to join the "radiologic follow-up":
- 45 years or 10 years younger than the youngest index case of pancreatic cancer in the family for familial cases
- 40 years or 5 years younger than the youngest index case of pancreatic cancer for subjects suffering from hereditary/genetic pancreatitis, Lynch syndrome, or carrying a known BRCA 1/2, PALB2, p16 genetic mutation with familiarity for pancreatic cancer
- 30 years for subjects suffering from FAMMM, Peutz-Jeghers syndrome
Exclusion Criteria:
- pregnancy