RT Book, Section
A1 Doherty, Gerard M.
SR Print(0)
ID 58095230
T1 Multiple Endocrine Neoplasia Type 1 (MEN 1)
T2 Quick Answers Surgery
YR 2010
FD 2010
PB The McGraw-Hill Companies
PP New York, NY
SN 
LK accesssurgery.mhmedical.com/content.aspx?aid=58095230
RD 2024/04/20
AB •  Also  known  as  Wermer  syndrome•  Characterized  by  tumors  of  the  parathyroid,  anterior  pituitary,  and  pancreas•  Tumors  may  develop  synchronously  or  metachronously,  may  be  benign  or  malignant,  and  may  be  hyperplasia,  adenomas,  or  carcinoma•  Pancreatic  tumors  include  functioning  and  nonfunctioning  islet  tumors  such  as  gastrinoma,  insulinoma,  glucagonoma,  VIPoma,  somatostatinoma,  pancreatic  polypeptide  tumors  (PPomas)•  Other  tumors  in  MEN  1  can  include  adrenocortical  tumors,  thymic  or  bronchial  carcinoid  tumors,  multiple  lipomas,  cutaneous  angiofibromas  and  collagenomas•  Syndrome  is  transmitted  as  autosomal  dominant  trait•  Most  common  mutation  is  in  the  menin  gene  on  11q13•  Trait  has  100%  penetrance  but  variable  expressivity