RT Book, Section
A1 Doherty, Gerard M.
SR Print(0)
ID 58095296
T1 Multiple Endocrine Neoplasia Type 2 (MEN 2)
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=58095296
RD 2024/04/19
AB •  Family  of  syndromes  including:  -MEN  2A  (Sipple  syndrome)-MEN  2B-Familial  medullary  thyroid  carcinoma  (FMTC)•  MEN  2A  is  characterized  by:  -Medullary  thyroid  carcinoma  (MTC)-Pheochromocytomas-Parathyroid  hyperplasia•  MEN  2B  consist  of  MTC,  pheochromocytoma,  mucosal  neuromas,  gangliomatosis  of  the  GI  tract,  and  a  distinctive  marfinoid  habitus-Also  have  high  incidence  of  skeletal  abnormalities,  such  as  congenital  dislocation  of  the  hip,  pes  planus  or  cavus,  kyphosis,  and  pectus  excavatum•  Transmitted  as  mendelian  autosomal  trait,  but  can  occur  de  novo  (especially  MEN  2B)•  Common  mutation  is  in  the  Ret  proto-oncogone  (tyrosine  kinase),  which  maps  to  the  centromeric  region  of  chromosome  10•  Families  with  hereditary  MTC  need  to  be  identified  with  an  aggressive  screening  program  because  early  diagnosis  and  thyroidectomy  renders  MTC  curable  in  a  large  percentage  of  patients