A%s"rac" . I'"rod(c"o') Rha)doyosar"oa is the ost "oon so#t*tissue sar"oa in "hildhood, representing +, o# all "hildhood "an"ers . -t is thought arising #ro priiti%e esen"hyal "ells "oitted to s.eletal us"le di##erentiation and "an o""ur in a %ariety o# organs and tissues, in"luding those that la". striated us"le./here are three su)groups o# rha)doyosar"oa 0 e)ryonal 1+2*32,4, al%eolar 115*6+,4 and pleoorphi" 1+*12,4. E)ryonal and al%eolar rha)doyosar"oas o""ur priarily in "hildren, and pleoorphi" rha)doyosar"oa o""urs ostly in adults. Care#ul diagnosti" iaging o# potential disease sites at the tie o# presentation is "ru"ial in assigning ris.*)ased therapy.
Case *rese'"a"o') A *17 year old girl presented with right lower leg rapidly growing asses. 8ast edi"al history was unrear.a)le. !aily history is negati%e #or "an"er, physi"al e9a nota)le #or non*tender right lower leg swelling and soe ne"roti" areas. :iopsy results were al%eolar rha)doyosar"oa. Chest 9*ray and a)doinal ultrasound results were no etastati" lesion. 8lain radiograph o# right lower leg showed so#t tissue ass with poorly de#ined argins and lo"al )one in%asion o# the #i)ula with pereati%e pattern o# )one destru"tion and interrupted periosteal rea"tion. !indings on ultisli"e C/ were dilatation o# the soleus us"les with a poorly de#ined so#t tissue ass surrounding id and distal #i)ula. /he #i)ula is eroded, shows tuor in%asion o# the adja"ent )one. A#ter intra%enous "ontrast ediu adinistration, the tuor shows inhoogenous enhan"eent representing ne"roti" areas. 8atient and parents re#used surgi"al treatent 1aputation4. E9ternal radiation with Co)alt ;2, total dose ;2 (y, in 72 #ra"tion)
Dsc(sso') Diagnosis o# rha)doyosar"oa is )ased on ananesis, physi"al e9aination, iaging and histopathology. Ananesis and physi"al e9aination o# this 17 year old girl lead to alignan"y "ondition. :iopsy results were al%eolar rha)doyosar"oa. So#t tissue ass with poorly de#ined argin is %isi)le on plain 9*ray wih erosion and destru"tion o# #i)ula. /he odality o# "hoi"e #or diagnosis o# so#t tissue sar"oas is MR-. Sin"e no MR- is a%aila)le in the #a"ility, there#ore this patient was sent to attend ultisli"e C/ #or e"aluation o# the e9tent o# tuor. /his patient had radiation without surgi"al e9"ision )e"ause she and her parents re#used surgi"al treatent. /he radiation responses were poor .
Co'cl(so') Al%eolar rha)doyosar"oa o""urs priarily in "hildren o# all ages, #re<uently on the e9treities. =esion is o#ten present as rapidly growing asses. /his tuor "ontains round or o%al "ells with priiti%e yo)lasti" #eatures. :y using MR-, the iages are ore "lear to show "ontrast )etween tuor with adja"ent stru"tures, also pro%ides e9"ellent three* diensional de#inition o# #as"ial planes. >hene%er MR- "annot )e per#ored, ultisli"e C/ "an )e used as alternati%e iaging in deterining tuor in%ol%eent o# us"le, )one, joints, or neuro%as"ular stru"tures. Al%eolar rha)doyosar"oa is one o# high*grade tuors that ore aggressi%e than the e)ryonal type. /reatent usually "onsists o# surgi"al e9"ision and&or post operati%e "heoterapy and radiation.