Pancreatic cancer, a common cancer in the U.S., has historically been associated with a poor outcome.

Epidemiology

• Incidence - 9-10 cases per 100,000
• Age - peak incidence 60-70 years
• Sex - M:F, 1:1.3
• Ethnicity - 30-40% higher rate in African-Americans

Risk Factors

• Low socioeconomic status
• Male gender
• Tobacco abuse
• Presence of chronic disease states
  • Chronic pancreatitis
  • Diabetes mellitus
  • Prior cholecystectomy
• Occupational exposures
  • DDT, benzidine, dry cleaning agents, polychlorinated biphenyls
• Genetics
  • Increased risk if family history of pancreatic cancer
  • Familial syndromes - BRCA 1 and 2 mutations, familial multiple mole melanoma syndrome, Peutz Jeghers syndrome, hereditary nonpolyposis, colorectal cancer, ataxia-telangiectasia syndrome, hereditary pancreatitis syndrome, VHL, Li-Fraumeni syndrome, familial pancreatic cancer

Pathophysiology

• Tumor is usually ductal adenocarcinoma
• Also have mucinous cystadenocarcinomic variants
• Rare neuroendocrine tumors also affect the pancreas
  • Insulinoma
  • Glucagonoma
• 70-80% of tumors are located in head of the pancreas

Clinical Presentation

• No specific early warning symptoms
• Usually abdominal pain and weight loss
• Obstructive jaundice if tumor is at the head of the pancreas
• Late features - ascites, abdominal mass

Diagnosis

• Indications for testing
  • Patient presents with jaundice and pancreatic mass
  • Monitoring for tumor recurrence after surgery
• Laboratory testing
  • CA 19-9 serum antigen testing - sensitivity depends on stage of cancer (70-90% sensitivity and 90% specificity)
    • May be elevated in benign obstructive jaundice, chronic pancreatitis
    • Should be used in conjunction with imaging studies to diagnose pancreatic cancer
    • Limited use as early screening
  • Serial monitoring recommended to assess follow up after potentially curative surgery or response to palliative chemotherapy
  • Other potential markers include MUC-1 antigen (CA15-3 antigen) and CEACAM1 (Carcinoembryonic antigen-related cell adhesion molecule 1) - neither has been sufficiently validated
  • Cystic lesions - fluid concentrations of amylase, CEA antigen, and CA 19-9
    • Amylase <250 U/mL, CEA >800 ng/mL, CA 19-9 >37 U/mL highly suggestive of diagnosis
• Histology
  • Biopsy of tumor with histologic evaluation
  • FNA may result in intraperitoneal seeding
• Imaging Studies
  • Transabdominal ultrasound/CT/MRI
  • Endoscopic retrograde cholangiopancreatography (ERCP) to outline extent of ductal involvement