RT Book, Section
A1 Thakker, R. V.
A2 Jameson, J. Larry
A2 Fauci, Anthony S.
A2 Kasper, Dennis L.
A2 Hauser, Stephen L.
A2 Longo, Dan L.
A2 Loscalzo, Joseph
SR Print(0)
ID 1183992396
T1 Multiple Endocrine Neoplasia
T2 Harrison's Principles of Internal Medicine, 20e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259644016
LK accesspharmacy.mhmedical.com/content.aspx?aid=1183992396
RD 2024/04/19
AB Multiple  endocrine  neoplasia  (MEN)  is  characterized  by  a  predilection  for  tumors  involving  two  or  more  endocrine  glands.  Four  major  forms  of  MEN  are  recognized  and  referred  to  as  MEN  types  1–4  (MEN  1–4)  (Table  381-1).  Each  type  of  MEN  is  inherited  as  an  autosomal  dominant  syndrome  or  may  occur  sporadically,  that  is,  without  a  family  history.  However,  this  distinction  between  familial  and  sporadic  forms  is  often  difficult  because  family  members  with  the  disease  may  have  died  before  symptoms  developed.  In  addition  to  MEN  1–4,  at  least  six  other  syndromes  are  associated  with  multiple  endocrine  and  other  organ  neoplasias  (MEONs)  (Table  381-2).  These  MEONs  include  the  hyperparathyroidism-jaw  tumor  (HPT-JT)  syndrome,  Carney  complex,  von  Hippel-Lindau  disease  (Chap.  380),  neurofibromatosis  type  1  (Chap.  86),  Cowden’s  syndrome  (CWS),  and  McCune-Albright  syndrome  (MAS)  (Chap.  405);  all  of  these  are  inherited  as  autosomal  dominant  disorders,  except  for  MAS,  which  is  caused  by  mosaic  expression  of  a  postzygotic  somatic  cell  mutation  (Table  381-2).