CS1LCSAkCCMA

CsLeosarcoma ls a cancerous Lumor LhaL occurs ln Lhe bone MosL osLeosarcomas appear ln Lhe
long bones of Lhe body such as Lhe femur (Lhe Lhlgh bone)Lhe Llbla (Lhe shlnbone) or Lhe humerus
(Lhe bone LhaL runs from Lhe shoulder Lo Lhe elbow)alLhough Lhey can appear ln any bones
CsLeosarcoma ls Lhe mosL common Lype of bone cancer and Lhe slxLh mosL common Lype of cancer ln chlldren
AlLhough oLher Lypes of cancer can evenLually spread Lo parLs of Lhe skeleLon osLeosarcoma ls one of Lhe few LhaL
acLually beglnln bones and someLlmes spread (or meLasLaslze) elsewhere usually Lo Lhe lungs or oLher bones
8ecause osLeosarcoma usually develops from osLeoblasLs (Lhe cells LhaL make growlng bone) lL mosL commonly
affecLs Leens LhaL are experlenclng a growLh spurL 8oys are more llkely Lo have osLeosarcoma Lhan glrls and mosL
cases of osLeosarcoma lnvolve Lhe knee MosL osLeosarcomas arlse from random and unpredlcLable errors ln Lhe unA
of growlng bone cells durlng Llmes of lnLense bone growLh 1here currenLly lsnL an effecLlve way Lo prevenL Lhls
Lype of cancer 8uL wlLh Lhe proper dlagnosls and LreaLmenL a paLlenL wlLh osLeosarcoma do recover
nIS1Ck
CsLeosarcoma ls an anclenL dlsease LhaL ls sLlll lncompleLely undersLood 1he Lerm sarcoma was
lnLroduced by Lhe Lngllsh surgeon !ohn AbernaLhy ln 1804 and was derlved from Creek rooLs
meanlng fleshy excrescence

ln 1803 Lhe lrench surgeon Alexls 8oyer (personal surgeon Lo
napoleon) flrsL used Lhe Lerm osLeosarcoma 8oyer reallzed LhaL osLeosarcoma ls a dlsLlncL enLlLy
from oLher bone leslons such as osLeochondromas(exosLoses)
Lvldence of furLher organlzed LhoughL and purposeful lnvesLlgaLlon regardlng Lhls dlsease was
found by Lhe mld 1800s elLler recorded LhaL ln 1847 Lhe 8aron Culllaume uupuyLren
demonsLraLed hls lnLlmaLe knowledge of Lhe gross paLhologlc appearance of osLeosarcoma when
he wroLe Lhe followlng
CsLeosarcoma whlch ls a Lrue cancerous degeneraLlon of bone manlfesLs lLself ln Lhe form of a
whlLe or reddlsh mass lardaceous and flrm aL an early sLage of Lhe dlsease buL presenLlng aL a laLer
perlod polnLs of sofLenlng cerebrlform maLLer exLravasaLlng blood and whlLe or sLraw colored
fluld of a vlscld conslsLence ln lLs lnLerlor
under Lhe ausplces of Lhe Amerlcan College of Surgeons LrnesL Amory Codman (along wlLh !ames
Lwlng and !oseph 8loodgood) creaLed Lhe 8eglsLry of 8one Sarcoma ln 1921

1hls was a slgnlflcanL
sLep forward ln sLudylng Lhese rare and omlnous Lumors as lndlvldual surgeons had only llmlLed
experlence Lo gulde Lhem
AnoLher ma[or lnsLlLuLlon LhaL began Lo Lake shape ln Lhe early 1900s was Lhe 8lzzoll lnsLlLuLe ln
8ologna lLaly 1hls lnsLlLuLe whose bone Lumor rooLs were nurLured by vlLLorlo uLLl (18801940)
prospered under Lhe laLer guldance of persons such as ScaglleLLl and Campanaccl

Ma[or
conLrlbuLlons from Lhls lnsLlLuLlon have lncluded lnnovaLlve LreaLmenL for unlcameral bone cysLs
(ScaglleLLl) and lnLense sLudy of osLeoflbrous dysplasla (Campanaccl Lumor)
8y Lhe mld 1900s greaL sLrldes were belng made ln Lhe unlLed SLaLes ln Lhe fleld of bone paLhology
by Penry L !affe (18961979) and hls colleague Louls LlchLensLeln (19061977) Lach of Lhese men
publlshed LexLbooks devoLed Lo bone paLhology !affe ls also ofLen credlLed wlLh brlnglng order Lo
Lhe chaos LhaL was orLhopedlc paLhology 1ogeLher !affe and LlchLensLeln esLabllshed vlrLually all of
Lhe key hlsLologlc crlLerla LhaL are used Lo dlagnose mosL of Lhe commonly encounLered bone
Lumors
A dlfferenL ur !affe (norman !affe) along wlLh oLher researchers helped expand Lhe use of a varleLy
of effecLlve chemoLherapeuLlc agenLs ln Lhe 1970s and early 1980s

noL Lhe leasL of Lhese agenLs
were Adrlamycln and meLhoLrexaLe 1hese medlcaLlons (and oLhers LhaL followed) dramaLlcally
lmproved Lhe LreaLmenL of paLlenLs wlLh osLeosarcoma Lhrough Lhelr ablllLy Lo LreaL Lhe
mlcromeLasLaLlc dlsease LhaL was LhoughL Lo be presenL ln approxlmaLely 80 of paLlenLs

1hese
drugs were found Lo be useful boLh preoperaLlvely and posLoperaLlvely ln paLlenLs wlLh
osLeosarcoma a dlscovery made aL Lhe SloankeLLerlng Memorlal Cancer CenLer somewhaL
serendlplLously whlle cusLommade prosLheses were belng fabrlcaLed for paLlenLs awalLlng
surgery

Such preoperaLlve use of chemoLherapy came Lo be referred Lo as neoad[uvanL
chemoLherapy
An orLhopedlc surgeon from Calnesvllle llorlda Wllllam l Lnneklng Mu lnLroduced hls surglcal
sLaglng sysLem for musculoskeleLal sarcomas 1hls sLaglng sysLem helped organlze Lhe orLhopedlc
surglcal approach Lo boLh blopsy and deflnlLlve Lumor resecLlon for osLeosarcoma as well as for
oLher musculoskeleLal sarcomas ur Lnneklngs lnfluence exLended far beyond hls sLaglng sysLem
because of hls lnLense commlLmenL Lo educaLlng oLhers regardlng musculoskeleLal Lumors Pe has
educaLed numerous orLhopedlc oncology fellows publlshed numerous research arLlcles and
conLlnued Lo conducL a yearly conLlnulng medlcal educaLlon course focuslng on benlgn and
mallgnanL Lumors
Causes
unknown
unA muLaLlon elLher lnherlLed or
acqulred afLer blrLh
lamlllal suscepLlblllLy
ln[ury
lnfecLlon
MeLabollc or hormonal dlsLurbance

k|sk and red|spos|ng Iactors
O 1he cause ls noL known
O ln some cases osLeosarcoma runs ln famllles and aL leasL one gene has been llnked Lo
lncreased rlsk
O 1hls gene ls also assoclaLed wlLh famlllal reLlnoblasLoma a cancer of Lhe eye LhaL occurs ln
chlldren resence of cerLaln rare lnherlLed cancers such as Lhe followlng
4 Lllraumenl syndrome a rare famlly predlsposlLlon Lo mulLlple Lypes of cancers
(such as sofL Llssue sarcomas breasL cancer braln Lumors osLeosarcoma leukemla
melanoma cancer of Lhe adrenal corLex and oLhers) caused by a muLaLlon ln a gene
Lhe p33 Lumorsuppressor gene LhaL normally curbs cancer
4 reLlnoblasLoma a mallgnanL Lumor of Lhe reLlna LhaL usually occurs ln chlldren
younger Lhan 4 years old
O Leenage growLh spurLs
O belng Lall for a speclflc age
O prevlous LreaLmenL wlLh radlaLlon for anoLher cancer especlally aL a young age or wlLh hlgh
doses of radlaLlon
O presence of a benlgn (noncancerous) bone dlsease
Symptoms of Csteosarcoma
1he mosL common sympLoms of osLeosarcoma are paln and swelllng ln legs or arms lL occurs mosL ofLen ln Lhe longer
bones of Lhe body such as above or below Lhe knee or ln Lhe upper arm near Lhe shoulder aln may be worse durlng
exerclse or aL nlghL and a lump or swelllng may develop ln Lhe affecLed area up Lo several weeks afLer Lhe paln sLarLs
aln LhaL perslsLenLly wakes Lhe paLlenL up aL nlghL or paln aL resL ls of parLlcular concern ln osLeosarcoma of Lhe leg
Lhe paLlenL may also develop an unexplalned llmp ln some cases Lhe flrsL slgn of Lhe dlsease ls a broken arm or leg
because Lhe cancer has weakened Lhe bone Lo make lL vulnerable Lo a break
D|agnos|ng Csteosarcoma
1o dlagnose osLeosarcoma Lhe docLor wlll llkely perform a physlcal exam obLaln a deLalled medlcal hlsLory and order
xrays Lo deLecL any changes ln bone sLrucLure 1he docLor may also order a magneLlc resoluLlon lmaglng (M8l)
scan of Lhe affecLed area whlch wlll flnd Lhe besL area Lo blopsy and show wheLher Lhe osLeosarcoma has spread
from Lhe bone lnLo nearby muscles and faL 1he docLor wlll also order a bone blopsy Lo obLaln a sample of Lhe Lumor
for examlnaLlon ln Lhe lab 1hls ls besL done by an orLhopedlc surgeon experlenced ln Lhe LreaLmenL of osLeosarcoma
(orLhopedlc oncologlsL)
SomeLlmes Lhe docLor does a needle blopsy uslng a long hollow needle Lo Lake a sample of Lhe Lumor A local
anesLhesla ls Lyplcally used ln Lhe area LhaLs belng blopsled AlLernaLlvely Lhe docLor may order an open blopsy ln
whlch a porLlon of Lhe Lumor ls removed ln Lhe operaLlng room by a surgeon whlle Lhe paLlenL ls under general
anesLhesla
lf a dlagnosls of osLeosarcoma ls made Lhe docLor wlll order C1 chesL scans as well as a bone scan and someLlmes
addlLlonal M8l sLudles 1hese wlll show lf Lhe cancer has spread Lo any parL of Lhe body beyond Lhe orlglnal Lumor
1hese LesLs wlll be repeaLed afLer LreaLmenL sLarLs Lo deLermlne how well lL ls worklng and wheLher Lhe cancer ls
conLlnulng Lo spread
athophys|o|ogy

CsLeoblasL
unA muLaLlon
MallgnanL osLeoblasL
rollferaLlon of mallgnanL osLeoblasL
lormaLlon of osLeold or lmmaLure bone
Slgns and sympLoms are Lhen observed
-urs|ng Intervent|ons
rovlde quleL envlronmenL and calm acLlvlLles Lo prevenL or lessen paln
rovlde comforL measure such as back rub change poslLlon and use of heaL or cold appllcaLlon
Lncourage dlverslonal acLlvlLles
AdmlnlsLer analgeslcs as lndlcaLed Lo maxlmal dose as needed
Lncourage Lhe paLlenL Lo lncrease fluld lnLake
Lncourage resL perlods Lo prevenL faLlgue
rovlde accuraLe lnformaLlon abouL Lhe slLuaLlon medlcaLlon and LreaLmenL
Assess muscle sLrengLh gross and flne moLor coordlnaLlon
rovlde plllows for cushlon and supporL
keep slde ralls up all Lhe Llme

1reat|ng Csteosarcoma
1reaLmenL of osLeosarcoma lncludes chemoLherapy(Lhe use of medlcal drugs Lo klll cancer cells and
shrlnk Lhe cancer) followed by surgery (Lo remove cancerous cells or Lumors) and Lhen more
chemoLherapy (Lo klll any remalnlng cancer cells and mlnlmlze chances of Lhe cancer comlng back)
Surgery ofLen can effecLlvely remove bone cancer whlle chemoLherapy can help ellmlnaLe
remalnlng cancer cells ln Lhe body
Surg|ca| 1reatment
Surglcal LreaLmenLs for osLeosarcoma conslsL of elLher ampuLaLlon or llmbsalvage surgery
CurrenLly paLlenLs wlLh osLeosarcomas lnvolvlng an arm or leg can be LreaLed wlLh llmbsalvage
surgery raLher Lhan ampuLaLlon ln llmbsalvage surgery Lhe bone and muscle affecLed by Lhe
osLeosarcoma are removed leavlng a gap ln Lhe bone LhaL ls fllled by elLher a bone grafL (usually
from Lhe bone bank) or more ofLen a speclal meLal prosLhesls 1hese can be approprlaLely maLched
Lo Lhe slze of Lhe bone defecL 1he rlsk of lnfecLlon and fracLure ls hlgher wlLh bank bone
replacemenL and Lherefore meLal prosLheses are more commonly used for reconsLrucLlon of Lhe
bone afLer removal of Lhe Lumor
lf Lhe cancer has spread Lo Lhe nerves and blood vessels surroundlng Lhe orlglnal Lumor on Lhe
bone ampuLaLlon (removlng parL of a llmb along wlLh Lhe osLeosarcoma) ls ofLen Lhe only cholce
When osLeosarcoma has spread Lo Lhe lungs or elsewhere surgery may also be performed Lo
remove Lumors ln Lhese dlsLanL locaLlons


Chemotherapy
ChemoLherapy ls usually glven boLh before and afLer surgery lL ellmlnaLes small pockeLs of cancer
cells ln Lhe body even Lhose Loo small Lo appear on medlcal scans A paLlenL wlLh osLeosarcoma ls
glven Lhe chemoLherapy drugs lnLravenously (Lhrough a veln) or orally (by mouLh) 1he drugs enLer
Lhe bloodsLream and work Lo klll cancer ln parLs of Lhe body where Lhe dlsease has spread such as
Lhe lungs or oLher organs
Short1erm and Long1erm S|de Lffects
AmpuLaLlon carrles lLs own shorLLerm and longLerm slde effecLs lL usually Lakes aL leasL 3 Lo 6
monLhs unLll a young person learns Lo use a prosLheLlc (arLlflclal) leg or arm and Lhls ls [usL Lhe
beglnnlng of longLerm psychologlcal and soclal rehablllLaLlon
WlLh a llmb salvage surgery one usually sLarLs bendlng Lhe knee or Lhe affecLed body parL almosL
lmmedlaLely A conLlnuous passlve moLlon (CM) machlne LhaL conLlnuously bends and sLralghLens
Lhe knee may be used Lo lmprove moLlon for Lumors around Lhe knee hyslcal Lherapy and
rehablllLaLlon for 6 Lo 12 monLhs followlng surgery usually enables Lhe paLlenL Lo walk lnlLlally wlLh
a walker or cruLches and Lhen wlLhouL any asslsLlve devlces Larly compllcaLlons afLer surgery
lnclude lnfecLlon and slow heallng of Lhe surglcal wound and Lhe meLal prosLheLlc devlce or Lhe
bank bone may need Lo be replaced ln Lhe long Lerm CLher laLe problems mlghL lnclude fracLure of
Lhe bank bone or fallure of Lhe bank bone Lo heal Lo Lhe paLlnLs bone whlch mlghL requlre more
surgery
Many of Lhe medlcaLlons used ln chemoLherapy also carry Lhe rlsk of boLh shorLLerm and longLerm
problems ShorLLerm effecLs lnclude anemla abnormal bleedlng and lncreased rlsk of lnfecLlon
due Lo desLrucLlon of Lhe bone marrow as well as kldney damage and mensLrual lrregularlLles
Some drugs carry a rlsk of bladder lnflammaLlon and bleedlng lnLo Lhe urlne hearlng loss and llver
damage CLhers may cause hearL and skln problems ?ears afLer chemoLherapy for osLeosarcoma
paLlenLs have an lncreased rlsk of developlng oLher cancers
Chances for a Cure
8ecenL sLudles have reporLed LhaL survlval raLes of 60 Lo 80 are posslble for osLeosarcoma LhaL
hasnL spread beyond Lhe Lumor dependlng on Lhe success of chemoLherapy
CsLeosarcoma LhaL has spread cannoL always be LreaLed as successfully Also a paLlenL whose
osLeosarcoma ls locaLed ln an arm or leg generally has a beLLer prognosls Lhan one whose dlsease
lnvolves Lhe rlbs shoulder blades splne or pelvlc bones
-ew 1reatments
1reaLmenLs are belng developed and researched wlLh new chemoLherapy drugs CLher research ls
focused on Lhe role cerLaln growLh facLors mlghL play ln Lhe developmenL of osLeosarcoma 1hls
research may be used Lo develop new medlcaLlons Lo slow Lhese growLh facLors as a way Lo LreaL
Lhe cancer
lor osLeosarcomas LhaL cannoL be removed surglcally sLudles are now underway Lo LesL LreaLmenLs
LhaL use new comblnaLlons of chemoLherapy and locallzed hlghdose radlaLlon
Comp||cat|ons
(After surgery)
lnfecLlon
Slow heallng of Lhe surglcal wound
Lhe meLal prosLheLlc devlce or Lhe
bank bone may need Lo be replaced ln
Lhe long Lerm
fracLure of Lhe bank bone
fallure of Lhe bank bone Lo heal Lo Lhe
paLlenLs bone whlch mlghL requlre
more surgery
(Chemotherapy)
Anemla
Abnormal bleedlng
lncreased rlsk of lnfecLlon
kldney damage
MensLrual lrregularlLles
8lsk of bladder lnflammaLlon
Pearlng loss
Llver damage
PearL and skln problems


kLAkLD 8
CnAVL2 kA1LLL-L 8
CCkUS kLGI-L A-GLLA 8
GkCU 10 S8C CC-
200M 600M