RT Book, Section
A1 Morita, Shane Y.
A1 Dackiw, Alan P.B.
A1 Zeiger, Martha A.
A2 Morita, Shane Y.
A2 Dackiw, Alan P. B.
A2 Zeiger, Martha A.
SR Print(0)
ID 6163328
T1 Chapter 15. Pheochromocytoma and Paraganglioma
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=6163328
RD 2024/04/19
AB Pheochromocytomas  and  paragangliomas,  otherwise  referred  to  as  extra-adrenal  pheochromocytomas,  are  rare  neuroendocrine  tumors  that  originate  from  neural  crest  cells  of  the  autonomic  nervous  system.1  They  secrete  catecholamines  in  variable  amounts  (e.g.,  those  of  the  head  and  neck  produce  less  than  those  located  within  the  abdomen).1  Sympathetic  paraganglia  are  intimately  associated  with  the  adrenal  medulla  and  organ  of  Zuckerkandl,  and  parasympathetic  paraganglia  are  associated  with  the  carotid  bodies.2  Neural  crest  tumors  that  arise  from  the  adrenal  medulla  are  referred  to  as  pheochromocytomas,  and  those  that  occur  extra-adrenally  are  called  paragangliomas.  In  2004,  the  World  Health  Organization  (WHO)  clarified  the  definition  of  pheochromocytomas  as  tumors  that  arise  in  the  adrenal  medulla  and  that  are  derived  from  chromaffin  cells  of  neural  crest  origin.3  Pheochromocytomas  were  first  described  in  1886  by  Felix  Fränkel.4  Evidence  suggests  that  the  patient  described,  Ms.  Minna  Roll,  had  bilateral  adrenal  lesions  and  multiple  endocrine  neoplasia  type  2  (MEN2).  This  is  based  on  genetic  analyses  performed  in  2007.5