1. Nat. Rev. Endocrinol. doi:10.1038/nrendo.2018.3
Figure 2 Distribution of endocrine and non-endocrine tumours in patients with MEN1
Figure 2 | Distribution of endocrine and non-endocrine tumours in patients with MEN1. a | Patients with multiple endocrine neoplasia type 1 (MEN1) might develop endocrine tumours involving the parathyroids (red), pancreas, pituitary, adrenal cortex and medulla, gastrointestinal tract, thymus, bronchial tree or non-endocrine tumours, such as facial angiofibromas, lipomas and collagenomas (which can arise anywhere in the body) and meningiomas. b | Frequencies of MEN1‑associated tumours are shown. The most common endocrine tumours in patients with MEN1 are parathyroid adenomas (>95% of patients). Pancreatic neuroendocrine tumours (PNETs) occur in 50–70% of patients (~40% gastrinomas, ~10% insulinomas, <1% glucagonomas, <1% vasoactive intestinal peptide (VIP)-secreting tumours (VIPomas) and ~20–50% pancreatic-polypeptide (PP) secreting tumours (PPomas) or non-functioning tumours). Anterior pituitary tumours occur in 20–40% of patients (~20% prolactinomas, ~10% somatotrophinomas, <5% corticotropinomas and ~5% non-functioning tumours), and adrenal tumours occur in 20–40% of patients (~40% cortical adenomas, which are usually non-secreting but might occasional secrete glucocorticoids or aldosterone, causing Cushing or Conn syndrome, respectively, and <1% phaeochromocytoma tumours arising from the medulla). The most common non-endocrine tumours in patients with MEN1 are angiofibromas (0–85%), collagenomas (0–70%) and lipomas (~30%). c | MRI transverse section of multiple PNETs (indicated by white arrows) in a patient with MEN1 is shown. d | MRI transverse section of a non‑MEN1 PNET (indicated by a white arrow) is shown.
Frost, M. et al. (2018) Current and emerging therapies for PNETs in patients with or without MEN1
Nat. Rev. Endocrinol. doi: /nrendo