Care for patients with multiple endocrine neoplasia type 1: the current evidence base

Care for patients with multiple endocrine neoplasia type 1: the current evidence base.

-Pieterman CRC, et al. Familial Cancer, Volume 10, Number 1 (2011), 157-171, DOI: 10.1007/s10689-010-9398-6.

• "The endocrine manifestations of MEN1 cannot be viewed upon as coinciding sporadic tumors."
• "In conclusion we state that the care for MEN1 patients is complex and should be provided by a centre of expertise. With the endocrinologist as primary caregiver, all important decisions should be made in a regular meeting of a multidisciplinary team, comprising of an endocrinologist, endocrine surgeon, radiologist, specialist nuclear medicine and paediatrician; if necessary expanded with a neurosurgeon, (radiation) oncologist, pathologist and clinical geneticist."
• Recommended protocol for periodic screening (summarized for patients at least 15 years of age):

  facility	frequency	treatment
  outpatient clinic	Every 2 years	History and physical exam
  labs	Every 2 years	Ionized calcium, chloride, phosphate, parathyroid hormone, fasting glucose, fasting insulin, fasting c-peptide, glucagon, fasting gastrin, pancreatic polypeptide, prolactin, insulin-like growth factor 1, platelet serotonin, chromogranin A
  imaging	Every 2 years	MRI of upper abdomen
  	Every 2-3 years	MRI of pituitary (intravenous contrast with gadolinium
  	Every 3-5 years	CT of thorax