TY  - CHAP
M1  - Book, Section
TI  - Ewing Sarcoma and Primitive Neuroectodermal Tumors
A1  - DuBois, Steven G.
A1  - Yustein, Jason T.
A2  - Kline, Mark W.
PY  - 2018
T2  - Rudolph's Pediatrics, 23e
AB  - Ewing  sarcoma  was  first  described  in  1921  by  James  Ewing  as  a  radiosensitive  malignancy  of  the  bone.  Since  that  initial  description,  insights  into  the  biology  of  this  tumor  have  expanded  the  definition  of  this  disease.  Based  upon  our  current  understanding,  Ewing  sarcoma  comprises  a  family  of  tumors  that  share  a  common  set  of  chromosomal  translocations  that  are  believed  to  be  the  originating  event  driving  the  pathogenesis  of  these  tumors  (see  below).  Ewing  sarcomas  may  arise  in  the  bone  or  soft  tissues  throughout  the  body.  They  may  be  undifferentiated  (pathologically  termed  Ewing  sarcoma)  or  show  evidence  of  neural  differentiation  (pathologically  termed  primitive  neuroectodermal  tumor,  or  PNET).  Moreover,  a  subset  of  these  tumors  that  arise  in  the  chest  wall  have  historically  been  referred  to  as  Askin  tumors.  As  Ewing  sarcoma,  PNET,  and  Askin  tumors  are  now  understood  to  share  the  same  fundamental  biology,  the  principles  that  drive  the  management  of  patients  with  these  tumors  are  largely  identical.  Therefore,  except  where  relevant  differences  exist,  this  chapter  will  refer  to  these  tumors  simply  as  Ewing  sarcoma.  This  is  in  contradistinction  to  a  more  recently  recognized  group  of  tumors  that  have  been  classified  as  Ewing-like  sarcomas.  These  tumors  harbor  different  genetic  translocations  and  are  biologically  distinct  from  Ewing  sarcoma,  and  little  is  known  about  their  optimal  management.  Further  discussion  of  Ewing-like  sarcomas  is  outside  the  scope  of  this  chapter.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1182911351
ER  -