The Cancer of the Pancreas Screening-5 CAPS5)Study

Study Overview
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Brief Summary
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Johns Hopkins clinical research office quality assurance group will monitor and audit this study at Johns Hopkins. The Sub Investigator at each site will be responsible for internal monitoring at their site.

Detailed Description
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The Sub Investigator at each site will be responsible for internal monitoring at their site. The site sub Investigator and study team will report any serious adverse events to Principal Investigator and annually report adverse events.

Official Title
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The Cancer of the Pancreas Screening-5 CAPS5)Study

Conditions
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Pancreas Cancer, Peutz-Jeghers Syndrome (PJS), Gene Mutation, Germline Mutation Carrier, Lynch Syndrome

Intervention / Treatment
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* Drug: Secretin
* Diagnostic Test: MRI
* Other: Tumor marker gene test with CA19-9


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Hereditary Pancreatitis or Peutz-Jeghers Syndrome or Strong family history of pancreas cancer on one side of the family tree or Confirmed germline mutation carrier (BRCA2, FAMMM, PALB2, BRCA1, HNPCC, PRSS1/2, or CTRC Endoscopic evaluation of pancreas scheduled Exclusion Criteria: Medical comorbidities or coagulopathy that contraindicate endoscopy Prior surgery that prevent optimal endoscopic ultrasound such as partial or complete gastrectomy with Bilroth or Roux-en-Y anastomosis Stricture or obstruction in the upper GI tract that does not allow passage of the echoendoscope Poor performance status Inability to provide informed consent Pregnancy.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Interventional Model Description: Evaluation of the effect of diagnostic tests for pancreatic cancer
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: Familial pancreas cancer relatives<br>High Risk Group 2 (familial pancreatic cancer relatives): > 55 years old or 10 years younger than the age of youngest relative with pancreatic cancer, and come from a family with 2 or more members with a history of pancreatic cancer (2 of which have a first-degree relationship consistent with familial pancreatic cancer), and have a first-degree relationship with at least one of the relatives with pancreatic cancer. If there are 2 or more affected blood relatives, at least 1 must be a first-degree relative of the individual being screened | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;Diagnostic Test: MRI<br>* MRI abdomen with contrast (MRCP) will be clinically indicated for abnormal novel CA-19-9 lab results.<br>* Other names: MRCP;Other: Tumor marker gene test with CA19-9<br>* A tumor marker gene test that will be used to stratify individuals into one of several circulating tumor marker reference ranges for CA19-9. The variants in the genes FUT3 and FUT2 affect the levels of CA19-9.<br>|
| Active Comparator: Group 1 germline mutation carrier<br>High Risk Group 3 (Group 1 germline mutation carriers with an associated with an estimated lifetime risk of pancreatic cancer of  10% or higher): a. > 50 years old or 10 years younger than the age of the youngest relative affected, if pancreatic cancer is in family, and b. The Patient is a carrier of a confirmed BRCA2, ATM or PALB2 mutation, regardless of family history of pancreatic cancer. b.> Individual is a carrier of a confirmed FAMMM (p16/CDKN2A) mutation, age 40 years or older, regardless of family history of pancreas cancer. | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;Diagnostic Test: MRI<br>* MRI abdomen with contrast (MRCP) will be clinically indicated for abnormal novel CA-19-9 lab results.<br>* Other names: MRCP;Other: Tumor marker gene test with CA19-9<br>* A tumor marker gene test that will be used to stratify individuals into one of several circulating tumor marker reference ranges for CA19-9. The variants in the genes FUT3 and FUT2 affect the levels of CA19-9.<br>|
| Active Comparator: Group 2 germline mutation carrier<br>High Risk Group 4 (Group 2 germline mutation carriers with an associated with an estimated lifetime risk of pancreatic cancer of  5%): > 50 years old or 10 years younger than the age of the youngest relative with pancreatic cancer, and The patient is a carrier of a confirmed BRCA1 or HNPCC (hereditary non-polyposis colorectal cancer or Lynch syndrome, hMLH1, hMSH2, PMS1, hMSH6, EpCAM) gene mutation, and there is > 1 pancreatic cancer in the family, one of whom is a first- or second-degree relative of the subject to be screened. | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;Diagnostic Test: MRI<br>* MRI abdomen with contrast (MRCP) will be clinically indicated for abnormal novel CA-19-9 lab results.<br>* Other names: MRCP;Other: Tumor marker gene test with CA19-9<br>* A tumor marker gene test that will be used to stratify individuals into one of several circulating tumor marker reference ranges for CA19-9. The variants in the genes FUT3 and FUT2 affect the levels of CA19-9.<br>|
| Active Comparator: Hereditary pancreatitis<br>High risk group 5 (hereditary pancreatitis) with confirmed gene mutations that predispose to chronic pancreatitis, such as PRSS1, PRSS2, CTRC) and age 50 years or older (these patients have an estimated lifetime risk for pancreatic cancer of 40%) or twenty-years since their first attack of pancreatitis, whichever age is younger. | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;Diagnostic Test: MRI<br>* MRI abdomen with contrast (MRCP) will be clinically indicated for abnormal novel CA-19-9 lab results.<br>* Other names: MRCP;Other: Tumor marker gene test with CA19-9<br>* A tumor marker gene test that will be used to stratify individuals into one of several circulating tumor marker reference ranges for CA19-9. The variants in the genes FUT3 and FUT2 affect the levels of CA19-9.<br>|
| Active Comparator: Peutz-Jeghers Syndrome<br>At least 30 years old, and at least 2 of 3 criteria diagnostic of Peutz-Jeghers syndrome (characteristic intestinal hamartomatous polyps, mucocutaneous melanin deposition, or family history of Peutz-Jeghers syndrome), or, known STK11 gene mutation carrier | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;Diagnostic Test: MRI<br>* MRI abdomen with contrast (MRCP) will be clinically indicated for abnormal novel CA-19-9 lab results.<br>* Other names: MRCP;Other: Tumor marker gene test with CA19-9<br>* A tumor marker gene test that will be used to stratify individuals into one of several circulating tumor marker reference ranges for CA19-9. The variants in the genes FUT3 and FUT2 affect the levels of CA19-9.<br>|
| Active Comparator: Negative control<br>are undergoing routine EGD or Colonoscopy; or Endoscopic Ultrasound (EUS) and/or Endoscopic Retrograde Cholangiopancreatography (ERCP) for non-pancreatic indications as part of their standard medical care, and have no clinical or radiologic suspicion of pancreatic disease (chronic pancreatitis or pancreatic cancer) | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;|
| Active Comparator: Chronic Pancreatitis<br>are undergoing EUS and/or ERCP for evaluation and/or treatment of suspected or proven chronic pancreatitis as part of their standard medical care, and, have no clinical or radiologic suspicion of pancreatic cancer | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;|
| Active Comparator: Pancreas cancer<br>a. are undergoing EUS and/or ERCP for evaluation and/or treatment of suspected or proven pancreatic ductal adenocarcinoma (based on clinical and radiologic evidence) | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;|
| Active Comparator: Pancreas cyst, IPMN evaluation<br>are undergoing EUS and/or ERCP for evaluation and/or treatment of suspected or proven pancreatic cancer precursor, intraductal papillary mucinous neoplasm (based on clinical presentation and radiologic or prior EUS or radiologic evidence of a dilated main pancreatic duct and/or pancreatic cystic lesion communicating with the pancreatic ductal system). | Drug: Secretin<br>* inject Secretin to stimulate pancreatic digestive fluid, which is collected in duodenum near ampulla via endoscope suction port. This fluid will be assessed for biomarkers.<br>* Other names: ChiRhoStim;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evaluate pancreatic juice for early cancer markers. | Aim #1: To evaluate pancreatic fluid mutations and circulating pancreatic epithelial cells as accurate markers of neoplasia by comparing their prevalence in cases with sporadic pancreatic neoplasia to healthy and disease controls. | 10 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Compare pancreas juice with pancreas cyst fluid | Aim #2: To compare the prevalence of pancreatic fluid mutations and circulating pancreatic epithelial cells among a prospective cohort of individuals with sporadic pancreatic cysts undergoing pancreatic surveillance. | 10 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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familial pancreas cancer, (Peutz-Jeghers Syndrome) PJS, Breast cancer (BRCA) 2, Partner and Locator of BRCA2 (PALB2), Familial Atypical Multiple Mole- Melanoma (FAMMM), p16, CDKN2A, Breast Cancer (BRCA)1, (hereditary non-polyposis colorectal cancer or Lynch syndrome) HNPCC, Lynch Syndrome, hereditary pancreatitis, Protease Serine (PRSS), Chymotrypsin C (CTRC), Ataxia Telangiectasia Mutated(ATM)