Neoplasms

Skin and Connective Tissue Diseases

All Conditions

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Diseases and Abnormalities at or Before Birth

Nutritional and Metabolic Diseases

Immune System Diseases

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Pancreatic Neoplasms

Melanoma

Pancreatitis

Pancreatitis, Chronic

Ataxia

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Telangiectasis

Ataxia Telangiectasia

Peutz-Jeghers Syndrome

Nevus

Colorectal Neoplasms

Colorectal Neoplasms, Hereditary Nonpolyposis

Gastrointestinal Neoplasms

Digestive System Neoplasms

Endocrine Gland Neoplasms

Gastrointestinal Diseases

Pancreatic Diseases

Endocrine System Diseases

Intestinal Neoplasms

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Intestinal Polyposis

Lentigo

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Pigmentation Disorders

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Hereditary Pancreatitis

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Amino Acids

Pancrelipase

Pancreatin

Secretin

Hormones

Hormone Antagonists

Serine

High Risk Group 2 (familial pancreatic cancer relatives):

1. \> 55 years old or 10 years younger than the age of youngest relative with pancreatic cancer, and
2. 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
3. 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

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.

High Risk Group 4 (Group 2 germline mutation carriers with an associated with an estimated lifetime risk of pancreatic cancer of \~5%):

1. \> 50 years old or 10 years younger than the age of the youngest relative with pancreatic cancer, and
2. 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.

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.

1. At least 30 years old, and
2. 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,
3. known STK11 gene mutation carrier

1. 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
2. have no clinical or radiologic suspicion of pancreatic disease (chronic pancreatitis or pancreatic cancer)

1. 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,
2. have no clinical or radiologic suspicion of pancreatic cancer

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)

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).

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.

MRI abdomen with contrast (MRCP) will be clinically indicated for abnormal novel CA-19-9 lab results.

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.

Tumor marker gene test with CA19-9

Hilary Cosby, RN

Scott Merenda, BSN

James Farrell, MD

Hilary Cosby, RN, CGRN

Michael Goggins, MD

Marcia I Canto, MD

Emily Blair

Chinedu Ukaegbu

Sapna Syngal, MD

Erika Koeppe, MS

Elena Stoffel, MD

Tiffany Lam

Fay Kastrinos, MD

Barbara Heaton

Wendy Brock, RN

Amitabh Chak, MD

Maureen DeMarshall, RN

Daniel Clay

Bryson Katona, MD

Christine Decapite

Sara Booz

Randall Brand, MD

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.

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.

Evaluation of the effect of diagnostic tests for pancreatic cancer

No masking of the diagnostic test results

The Cancer of the Pancreas Screening-5 CAPS5)Study

Aim #3: To determine the prevalence of pancreatic lesions, pancreatic fluid mutations and circulating pancreatic epithelial cells among a large cohort of high-risk individuals undergoing pancreatic screening and surveillance of a new cohort in which screening is begun at age \>55.

Aim #4 To evaluate the diagnostic performance of a tumor marker gene test to personalize the normal reference range of tumor markers such as CA19-9 for patients undergoing pancreatic surveillance.

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.

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.

ChiRhoClin, Inc.

National Cancer Institute (NCI)

National Institutes of Health (NIH)

Johns Hopkins University

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
The Cancer of the Pancreas Screening-5 CAPS5)Study	NCT02000089	Pancreas Cancer Peutz-Jeghers S... Gene Mutation Germline Mutati... Lynch Syndrome	Secretin MRI Tumor marker ge...	18 Years -	Johns Hopkins University
The Cancer of the Pancreas Screening-5 CAPS5)Study	NCT02000089	Pancreas Cancer Peutz-Jeghers S... Gene Mutation Germline Mutati... Lynch Syndrome	Secretin MRI Tumor marker ge...	18 Years -	Johns Hopkins University

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