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neo_pancreas

pancreatic cancer

introduction

  • “pancreatic cancer” usually refers to adenocarcinoma of the exocrine pancreatic ducts which accounts for 85% of all pancreatic neoplasia
  • 95% of all malignant pancreatic tumours arise from the exocrine pancreas
  • endocrine pancreas may give rise to pancreatic neuroendocrine tumours such as islet cell tumours

DDx of pancreatic mass on CT scan

cystic lesions

  • pancreatic true cysts
  • pancreatic retention cysts
  • pancreatic mucinous non-neoplastic cysts
  • lymphoepithelial cysts (rare)
  • pancreatic pseudocysts (walled off pancreatic fluid collections)
  • pancreatic cystic neoplasms:
    • intraductal papillary mucinous neoplasm of the pancreas
    • serous cystic tumors
    • mucinous cystic neoplasms
    • solid pseudopapillary neoplasms

solid lesions

  • exocrine pancreatic adenocarcinoma
  • islet cell tumour
  • lymphoma (rare)
    • suggested by B symptoms, raised LDH, normal serum CA 19-9, size > 6cm, surrounding enlarged LNs
  • metastatic cancer (rare)

exocrine pancreatic adenocarcinoma

overview

risk factors

  • overall population lifetime risk 1.3%
  • non-hereditary chronic pancreatitis
    • 26x relative risk over 7years
    • cumulative risk: ~2% at 10 years, ~4% at 20 years
  • genetics:
    • 5-10% have a 1st degree relative with exocrine pancreatic cancer
    • esp. if age < 50yrs
    • increased risk with:
      • familial pancreatic cancer
        • 2 or more 1st or 2nd degree family members ⇒ 18x relative risk
        • 3 or more family members ⇒ ~60x relative risk
      • hereditary breast cancer: BRCA and PALB2
      • p16, MLH1, MSH2, MSH6, or PMS2 mutations
      • Peutz-Jeghers syndrome
      • Familial atypical multiple-mole melanoma (FAMMM) syndrome
      • Ataxia-telangiectasia
      • Lynch syndrome and FAP
      • non-O blood groups:
        • blood gp A: 1.3x relative risk
        • blood gp AB: 1.5x relative risk
        • blood gp B: 1.7x relative risk
  • smoking
    • at least 1.5x relative risk
    • perhaps a 2x relative risk
  • obesity and lack of physical activity
    • BMI > 30 appears to have a 1.7x relative risk
  • height
    • appears to have an increased relative risk of 1.8x
  • diet, alcohol, caffeine - controversial, no clear significant risk evident on present data

clinical features

  • most patients present with:
    • anorexia, weight loss
    • epigastric pain
      • usually insidious onset with gnawing quality and may radiate to sides or back
      • often occurs even in small tumours
      • often helped by curling up into fetal position
      • rarely may present with pancreatitis if tumour obstructs pancreatic duct
  • half also present with:
    • nausea
    • back pain (especially with tumours arising in body or tail of pancreas)
  • some may present with progressive cholestatic jaundice (esp. if arises in pancreatic head, or late if due to liver mets), diarrhoea or vomiting
  • rarely may present with:
    • new onset atypical diabetes mellitus
    • nonbacterial thrombotic endocarditis and arterial emboli
    • pancreatic panniculitis, especially involving the legs, particularly in patients with the acinar cell variant of pancreatic cancer
    • cicatricial or bullous pemphigoid
  • may be discovered incidentally on CT scan (up to 7% of cases)
  • advanced disease is suggested by:
    • abdominal mass
    • Virchow's node (left supraclavicular lymphadenopathy)
    • Sister Mary Joseph's node periumbilical mass

spread

  • lymph nodes
  • liver
  • peritoneum
  • lungs
  • bone

prognosis

islet cell tumours

  • aka pancreatic neuroendocrine tumours (NET)
  • account for 3% of primary pancreatic tumours
  • can secrete various peptide hormones:
  • most are sporadic but some are due to hereditary conditions:
    • lifetime risk of developing a NET:
      • multiple endocrine neoplasia type I (MEN1) > 80%
      • von Hippel Lindau (VHL) syndrome < 20%
      • neurofibromatosis type I 10%
      • tuberous sclerosis 1%
neo_pancreas.txt · Last modified: 2014/04/03 18:53 (external edit)