RATIONALE: Screening for early pancreatic neoplasia may improve the ability to detect cancer in people who have a genetic risk for pancreatic cancer.

PURPOSE: Phase I trial to study the effectiveness of endoscopic ultrasound in screening for early pancreatic neoplasia in participants who have familial Peutz-Jeghers syndrome or relatives with familial pancreatic cancer.

OBJECTIVES:

• Determine the feasibility of using endoscopic ultrasonography (EUS) to screen for early pancreatic neoplasia, in terms of participant's willingness to undergo screening and safety and efficacy of the procedure, in participants with familial Peutz-Jeghers syndrome and relatives of patients with familial pancreatic cancer.
• Determine the prevalence and stage of early cancers in asymptomatic individuals (relative to that of symptomatic cancers diagnosed in kindreds) and the potential diagnostic yield of a screening program for familial pancreatic cancer and Peutz-Jeghers syndrome.
• Determine the EUS abnormalities and the phenotype of pancreatic cancer in high-risk individuals.
• Compare the EUS abnormalities in these participants with those in age-, race-, and sex-matched controls with and without chronic pancreatitis and sporadic pancreatic cancer, to determine which are specific and potentially diagnostic of early pancreatic neoplasia.
• Estimate the relative risk for pancreatic neoplasia adjusted for confounding factors in these participants.
• Correlate EUS and histologic abnormalities in these participants.
• Compare EUS accuracy for diagnosis of pancreatic neoplasia with cytology/histology in these participants.

OUTLINE: This is a pilot study.

All participants (including the control groups) undergo a complete history, endoscopic ultrasonography (EUS), serum trypsinogen analysis, and secretin-stimulated pancreatic juice collection.

High-risk participants (groups 1 and 2) also undergo a physical examination, genetic counseling, dual-phase contrast spiral abdominal CT scan, and serum CA 19-9 analysis. If the baseline EUS is abnormal, participants undergo EUS-guided fine needle aspiration (FNA) and endoscopic retrograde cholangiopancreatography. Pancreatic aspirates are graded for dysplasia. Participants diagnosed with a mass, cancer, or severe dysphagia are referred for surgery. EUS is performed on the resected specimen and adjacent normal tissue to directly correlate EUS abnormalities with histology in vitro.

Participants with a normal screening EUS and normal CT scan repeat EUS, CT scan, and serum CA 19-9 and trypsinogen analysis in 1 year. Participants with an abnormal EUS who do not have surgery and have EUS-guided FNA that does not show severe dysplasia or cancer repeat EUS/FNA and CT scan within 3-6 months.

PROJECTED ACCRUAL: A total of 60 high-risk participants (15 with Peutz-Jeghers syndrome [group 1] and 40 with familial pancreatic cancer relatives [group 2]) and 160 control participants (100 normal controls, 30 chronic pancreatitis controls, and 30 sporadic pancreatic cancer controls) will be accrued for this study within 1 year.

Ages Eligible for Study:  18 Years and above,  Genders Eligible for Study:  Both

Criteria

DISEASE CHARACTERISTICS:

• One of the following high-risk or control groups:
• Individuals with familial Peutz-Jeghers syndrome (high-risk group 1)
• At least 30 years old
• Characteristic intestinal hamartomatous polyps
• Mucocutaneous melanin deposition
• Relatives of familial pancreatic cancer patients (high-risk group 2)
• At least 40 years old OR 10 years younger than the youngest relative with pancreatic cancer
• 3 or more family members with a history of pancreatic cancer (2 of which have a first-degree relationship consistent with familial pancreatic cancer)
• First-degree relationship with at least 1 of the relatives with pancreatic cancer
• Negative controls (control group 1)
• Undergoing endoscopic ultrasonography (EUS) and/or endoscopic retrograde cholangiopancreatography (ERCP) for non-pancreatic indications as part of standard medical care
• No clinical or radiologic suspicion of pancreatic disease (i.e., chronic pancreatitis or pancreatic cancer)
• Individuals with chronic pancreatitis (control group 2)
• Undergoing EUS and/or ERCP for evaluation and/or treatment of suspected or proven chronic pancreatitis as part of standard medical care
• No clinical or radiologic suspicion of pancreatic cancer
• Individuals with sporadic pancreatic cancer (control group 3)
• Undergoing EUS and/or ERCP for evaluation and/or treatment of suspected or proven pancreatic ductal adenocarcinoma (based on clinical and radiological evidence)

PATIENT CHARACTERISTICS: Age

• 18 and over

Performance status

• Karnofsky 60-100%

Life expectancy

• Not specified

Hematopoietic

• No coagulopathy that would preclude endoscopy

Hepatic

• Not specified

Renal

• Not specified

Other

• No stricture or obstruction in the upper gastrointestinal tract that does not allow passage of echoendoscope
• No medical comorbidities that would preclude endoscopy

PRIOR CONCURRENT THERAPY: Biologic therapy

• Not specified

Chemotherapy

• Not specified

Endocrine therapy

• Not specified

Radiotherapy

• Not specified

Surgery

• No prior partial or complete resection of the pancreas
• No prior partial or complete gastrectomy with Billroth or Roux-en-Y anastomosis