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Periampullary cancer
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== 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 [[Category:Biliary]]
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