PanNETs

• Synonym: Islet cell tumours
• Epidemiology:

  • 2% of all pancreatic neoplasms

  • Most common in adults

  • 65-80% of PanNETs show aggressive behaviour (local invasion and/or distant metastases)

  • Clinical outcome correlates well with mitotic activity and Ki-67 proliferation index
• Pathogenesis:

  • Mutations of MEN1 gene

  • Loss-of-function mutations in tumour suppressor genes (e.g. PTEN, TSC2)

  • Inactivating mutations in ATRX and DAXX genes