RT Book, Section
A1 Grubbs, Elizabeth G.
A1 Perrier, Nancy D.
A1 Evans, Douglas B.
A1 Lee, Jeffrey E.
A2 Morita, Shane Y.
A2 Dackiw, Alan P. B.
A2 Zeiger, Martha A.
SR Print(0)
ID 6163449
T1 Chapter 16. Adrenocortical Carcinoma
T2 McGraw-Hill Manual: Endocrine Surgery
YR 2010
FD 2010
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-160645-5
LK accesssurgery.mhmedical.com/content.aspx?aid=6163449
RD 2024/04/19
AB Adrenocortical  carcinoma  (ACC)  is  a  rare  malignant  endocrine  neoplasm  with  an  estimated  incidence  of  0.5  to  2  cases  per  1  million  people  annually  in  the  United  States,1,2  accounting  for  0.02%  of  all  cancers  reported  annually.1  The  prognosis  for  most  patients  diagnosed  with  ACC  is  disappointingly  poor  because  of  delays  in  diagnosis  and  the  absence  of  effective  systemic  therapy.  Approximately  50%  of  affected  patients  do  not  survive  beyond  2  years  after  diagnosis,  and  the  5-year  mortality  rate  approaches  80%.3  ACC  has  a  bimodal  age  distribution  with  an  increased  incidence  among  children  younger  than  5  years  of  age  and  in  individuals  in  their  fourth  and  fifth  decades  of  life.  A  slightly  higher  incidence  rate  is  reported  for  women  than  for  men.4  Approximately  40%  of  ACCs  produce  clinically  significant  excess  amounts  of  steroid  hormones,  resulting  in  characteristic  signs  and  symptoms;5  female  patients  are  more  likely  to  have  an  associated  clinical  endocrine  syndrome.  Surgery  remains  the  only  effective  curative  treatment  for  ACC.  In  a  1996  study  of  risk  factors,  cigarette  smoking  and  the  use  of  oral  contraceptives  were  found  to  be  associated  with  the  development  of  ACC.6  An  association  has  also  been  described  between  ACC  and  congenital  adrenal  hyperplasia.7