Registry of Subjects at Risk of Pancreatic Cancer

Study Overview
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Brief Summary
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IRFARPC is a multicenter national registry designed to study the diagnosis and predisposing factors of subjects with an inherited increased risk for pancreatic cancer.

Detailed Description
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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.

Official Title
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Italian Registry of Families At Risk of Pancreatic Cancer

Conditions
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Familial Pancreatic Cancer, BRCA1 Mutation, BRCA2 Mutation, Lynch Syndrome, FAMMM - Familial Atypical Mole Malignant Melanoma Syndrome, Hereditary Pancreatitis, Peutz-Jeghers Syndrome

Intervention / Treatment
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* Radiation: MRCP
* Procedure: Endoultrasonography


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Familial pancreatic cancer relatives<br> | Radiation: MRCP<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>Procedure: Endoultrasonography<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>|
| Peutz-Jeghers syndrome<br> | Radiation: MRCP<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>Procedure: Endoultrasonography<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>|
| BRCA 1/2, PALB2, p16 mutations with familiarity for PC<br>Known genetic mutation and at least 1 1st- or 2nd-degree relative suffering from PC | Radiation: MRCP<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>Procedure: Endoultrasonography<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>|
| Lynch syndrome with familiarity for pancreatic cancer<br> | Radiation: MRCP<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>Procedure: Endoultrasonography<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>|
| FAMMM syndrome<br> | Radiation: MRCP<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>Procedure: Endoultrasonography<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>|
| Hereditary and genetic pancreatitis<br> | Radiation: MRCP<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>Procedure: Endoultrasonography<br>* Subjects fulfilling the radiological follow-up criteria will be submitted to MRCP or EUS according to the pancreatologist's prescription<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| This clinical study will assess the diagnostic yield of a clinical surveillance program for pancreatic neoplasia or predisposing conditions (PanIN, IPMNs) in high risk individuals | Number of malignant and pre-malignant lesions diagnosed over time | 25 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| This study will investigate possible risk factors for the incidence or the progression of for pancreatic neoplasia or predisposing conditions (PanIN, IPMNs) in high risk individuals | Identification of risk factors for malignant or pre-malignant lesions diagnosis | 25 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Familial Pancreatic Cancer, BRCA Mutation, Lynch Syndrome, Screening pancreatic cancer, Surveillance pancreatic cancer