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Conferences and Medical Updates

Familial Pancreatic Cancer

  • 2 or more patients with pancreatic cancer in family.
  • 10% of all pancreatic cancers are familial.
  • High risk FPC.  Families with > 3 members with cancer.
  • Hereditary Pancreatic cancer BRCA1/2, p16 mutations or FAMMM, Lynch syndrome, PJ syndrome, hereditary pancreatitis (cationic trypsinogen gene)
  • Hereditary breach and ovarian cancer HBOC BRCA 1 and BRCA2
  • PJ syndrome higher risk of colon cancer and should be monitored for pancreatic cancer also.  Moreover risk of gastric cancer, ovarian, breast, cervical and endometrial cancer
  • FAMM (familial atypical multiple mole melanoma). p16/CDKN2A gene.  They have atypical melanocytic nevi, autosomal dominant
  • Lynch – SB cancer, ureter cancer, renal pelvis cancer, brain, ovary and pancreatic cancer.  Age 50 1.3% and Age 70 4% risk of pancreatic cancer
  • HBOC syndrome – autosomal dominant.  Some patient family may have pancreatic cancer but no breast or ovarian cancer !!!!
  • BRCA mutation in Jewish patients.
  • PALB-2 Multiple family members with pancreatic cancer.  Also increased risk of breast cancer
  • Johns Hopkins, Mayo clinic, Creighton Univ, Mt Sinai, Anderson CC,  – sites for familial  cancers
  • Screening with EUS, dedicated MRI / MRCP or dedicated pancreatic protocol CT, aggressive evaluation of cysts. ????prophylactic pancreatectomy.
  • PanINs lesions – intraepithelial neoplasia (similar to colon cancer from adenoma).  High grade PanIN3 and more dense, different Kras mutations.
  • Thus 2 ways to develop pancreatic cancer PanIN or IPMN.
  • 3 D reconstruction for pancreatic neoplasm
  • MRI has no radiation advantage.
  • EUS not widely available and high inter observer variability.  Requires extra training.
  • Start screening at 50 or 10 years younger.  PJ start at 35