52 Multiple Endocrine Neoplasia
52.1 Type 1 MEN
• Hyperplasia of all four parathyroid glands
• Pancreatic islet tumours (including gastrinoma, insulinoma, glucagonoma, vasoactive intestinal peptide (VIP) tumour (VIPoma), or pancreatic polypeptide–producing tumour [PPoma])
• Anterior pituitary tumours (including prolactinoma, somatotropinoma, corticotropinoma, or non-functioning tumours)
• Other associated cutaneous tumours, including lipomas, angiofibromas, hypopigmented collagenomas
• 1 in 30,000 incidence
• Typically present in 2nd decade but diagnosis may be delayed for many years
• Cutaneous tumours may present first
• May be family history of skin lesions