RT Book, Section
A1 Thakker, Rajesh V.
A2 Longo, Dan L.
SR Print(0)
ID 1135230382
T1 Multiple Endocrine Neoplasia
T2 Harrison's Hematology and Oncology, 3e
YR 2016
FD 2016
PB McGraw-Hill Education
PP New York, NY
SN 9781259835834
LK hemonc.mhmedical.com/content.aspx?aid=1135230382
RD 2024/04/18
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  52-1).  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  52-2).  These  MEONs  include  the  hyperparathyroidism-jaw  tumor  syndrome,  Carney  complex,  von  Hippel-Lindau  disease  (Chap.  53),  neurofibromatosis  type  1  (Chap.  48),  Cowden’s  syndrome,  and  McCune-Albright  syndrome;  all  of  these  are  inherited  as  autosomal  dominant  disorders,  except  for  McCune-Albright  syndrome,  which  is  caused  by  mosaic  expression  of  a  postzygotic  somatic  cell  mutation  (Table  52-2).