Periampullary cancer

Four common types:

Pancreatic ductal adenocarcinoma (~80%)

• • Also most aggressive of these - only 15% of patients are surgical candidates at diagnosis
  • Among those who have surgery, median survival is 20 months, and 5 year survival of 20%
  • Commonly 50-70yo
  • RFs:
    • Smoking
    • Obesity
    • T2DM
    • Family history

Distal cholangiocarcinoma (~10%)

• • Distal CCA arises in CBD between junction of cystic duct and ampulla of Vater
  • RFs:
    • 60-70yo
    • PSC
    • Parasites
    • CP
    • HBV/HCV
    • Choledochal cysts
  • 5 year survival: 30% for local/regional disease, 2% for distant mets at time of diagnosis

Ampullary adenocarcinoma

• • Generally develop obstructive symptoms early, and therefore seen earlier
  • 80% resectable disease
  • There could be two different subtypes - intestinal (better) and pancreaticobiliary (worse survival)

Duodenal adenocarcinoma

• • Often quite large at diagnosis, because they can have unrestricted growth before symptoms are noted
• Rarely - neuroendocrine neoplasms, pancreatic cystic neoplasms, acinar and squamous cell carcinomas, GI stromal tumours, sarcomas, lymphomas, metastases.

Presentation

• Obstructive jaundice - often painless, but can have vague pain
• Cholangitis
• Pancreatic insufficiency
• GI bleeding

Clinically

• Look for sister mary joseph nodule or Virchow's node
• Sometimes have Courvoisier's sign - palpable non-tender GB

Investigation

• Bloods
  • INR
  • CA-19-9
• Imaging
  • CT is best first test if suspected, with pancreas protocol
  • PDAC best imaged PV phase
  • Double duct is most commonly a/w PDAC

Staging

• Stages I and II - Conventional resectability - localised, non-metastatic tumours without extension into major visceral vasculature
• Stage III - non-metastatic tumours with some degree of major vascular involvement
  • Abutment - <180deg loss of fat separation between tumour and vessel
  • Encasement >180deg
  • Divide stage III into:
    • 'Borderline resectable' - technically reconstructable vascular involvement

• 'Locally advanced unresectable'

• Stage IV - distant mets, unresectable

Tissue biopsy/stenting

• EUS + FNA

Treatment

• Resectable (stage I or II): upfront surgery followed by adjuvant CTX
  • Consider neoadjuvant - gaining traction recently
• Borderline resectable - Neoadjuvant CTX +/- RTX, then re-image, and assuming no progression, can be resected
• Locally advanced unresectable - CTX, and if it rarely downstages, it may become resectable later, but this is rare
• Unresectable (stage IV) - CTX

Surgery:

• Pancreaticoduodenectomy, either standard or pylorus-preserving - unclear which is superior. Minimally-invasive has equivalent oncologic results.
• Technique
  • Abdominal exploration - confirm no spread
  • Mobilisation of structures and formal tumour resection
  • Pancreaticobiliary and GI reconstruction