BONE TUMOURS - I & II - 2015.pptx

7/25/2019 BONE TUMOURS - I & II - 2015.pptx

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BONETUMOURSPROFESSOR.NYAN HTAIN

LINN


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Learning outcomes

At the end of the lecture the studentmust !e a!le to" E#$lain the $athogenesis of !one tumors and

tumor%li&e conditions.

'lassif( ma)or $rimar( tumors in*ol*ing!ones.

+escri!e the mor$holog( of !one tumors.

+escri!e the clinical course and distinctradiological features of !one tumors.

+iscuss the diagnosis and management !onetumors.


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Parts of a long bone


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BONE TUMOURS

Gross and morphologic appearances diverse.

Natural history varies from innocuous to rapidly fatal.

Classification mostly according to the normal cell or

tissue type they recapiculate.

Benign tumours greatly out number their malignant

counterparts and greatest frequency

in the first decade of life.Specific typesof tumours target certain age groups

and anatomic sites.

Location provides important diagnostic information.


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CLASSIFICATION OF BONETUMORS

Cartilage tumorsOsteocon!roma Con!romas Enchondroma

Periosteal chondroma Con!roblastoma Con!rom"#oi! $broma Con!rosarcoma +edi,erentiated

-esench(mal

'lear cell

Periosteal


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Osteogenic tumors

Osteoi! osteoma Osteoblastoma Osteosarcoma

'on*entional Teleangiectatic

Small cell

Lo grade central

Secondar( Parosteal

Periosteal

High grade surface


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Fibrogenic tumors Non%ossif"ing $broma Fibrous cortical !efect

Fibrosarcoma

Primiti&e Neuroecto!ermalTumor E'ing sarcoma

(iant Cell Tumor (iant cell tumor Malignant (iant cell tumor

Miscellaneous lesions

Aneur"smal bone c"st Fibrous !"s)lasia


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on!ar" bone neo)lasia is associated ith "

!one infarctschronic osteom(elitis

Pagets diseaseradiation

metal $rostheses

ndary neoplasms account for only a small fra


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CLINICAL FEATURES

)resentation &aries /benign lesions /0 fre1uentl(

as(mtomatic. incidental2nding. many /0%$roduce $ain. %slo groing mass. %

sudden $athologicfracture.

-limitation of mo*ement.

-general s(m$toms fe*er e#hausioneight% loss etc.


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BONE FORMIN( TUMOURS

$roduction ofbone!( the neo$lastic cells tumour !one usuall( de$osited as 'o&en

trabeculae *aria!l( minerali*e!

OSTEOMAS !osselated round to o*al sessile.

$ro)ect from sub)eriosteal surface ofcorte#+

most often arise on or insides,ulland

facial bones+


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multiple osteomas are seen in Gardnerssyndrome+

generall( slo' gro'ing tumours of littleclinicalsigni2cance.

the( consist of a com$osite of 'o&enand

lamellar !one fre1uentl( de$osited in cortical$attern.some *ariants contain a com$onent of

tra!ecular !one.

COMPLICATIONS . o!struction of a sinus ca*it(.


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OSTEOMA OF T/E S0ULL


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OSTEOMA


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OSTEOI1 OSTEOMA

benign!one tumour. less than2cm in greatest diameter. usuall( occur in teensand 23s3456 7

25"rs4 men " omen 5 2.8 can arise in any bone. )re!election/ a$$endicular s&eleton. $osterior elements of

s$ine. 536 of cases% femur orti!ia.

commonl( arise in corte#.

less fre1uentl( arise ithin medullar(


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Painis caused !( e#cessprostaglandin E2$roduced

!( $roliferating osteo!lasts.

MORP/OLO(:(ross

ell circumscri!ed round to o*al masses ofgritt( tan tissue.Microsco)iccom$osed of randoml( interconnecting

tra!eculae of 'o&en bone $redominentl(rimmed !( osteoblasts+surrounded !( loose connecti*e tissue

stroma


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uall( elicit a tremendous amount of reacti*ene formation that encircles the lesion.

1IO(RAP/ICALL:/ tumor manifests as smaln! lucenc" that ma( !e centrall" minerali*e!

i!us41uentl( treated !(ra!ioablation+


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Osteoi!osteoma

shoing randoml(oriented tra!eculae ofo*en !one rimmed

!( $rominentosteo!lasts.

The intertra!ecular

s$aces are 2lled !(*ascular looseconnecti*e tissue.


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OSTEOI1 OSTEOMA


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OSTEOBLASTOMA benign!one tumour si*e% larger than 2cm. in*ol*es the spinemore fre1uentl(.

CL9F/ dull aching $ain not res$onsi*e tosalic(lates.

/ISTOLO(IC FEATURES / identical to osteoidosteoma !ut usuall( does notinduce

mar&ed !on(reaction.

T9M% usuall( curetted or e#cised en!loc in aconser*ati*e fashion.

$ossi!ilit( of malignant change / remote


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OSTEOBLASTOMA


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OSTEOSARCOMA

malignant mesench(mal tumor inhich thecancerous cells produce bone

matrix.

6most common $rimar( malignant tumorof

!one.3


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SITE % usuall( arise in the meta)"sealregion of

long bones of e#tremities.

almost 536 occur about te ,nee an" bone can !e in*ol*ed. in $ersons 0 25"rs/ incidence in 8at!ones

and long !ones is almost e1ual.

PAT/O(ENESIS

!asic mechanisms that cause thede*elo$ment of osteosarcoma are still un&non.a$$ro#imatel( 436 ha*e ac1uired genetic

a!nor%


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tinoblastoma gene ?RB (ENE!" (ENE

defects in rband )5< $la( im$ortant roles.

!normalities inIN9:A hich encodes$;< 3 a cell(cle regulator4 and$;:3hich aids and a!ets $=>unction4are also seen in osteosarcoma.

steosarcomas tend to occur at sites of bone

rowth !ecause $roliferation ma&es osteo!las%c cells $rone to ac1uire mutations that couldad to malignant transformation.


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Maor sites of origins of Osteosarcomas


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MORP/OLO(:SUBT:PESare grouped according to"@SITE OF ORIGIN 3intramedullar( intracorticalor

surface4@DEGREE OF DIFFERENTIATION.@MUTIENTRIIT!3s(nchronous metachronous4@"RIMAR!3 underl(ing !one is unremar&a!le4 or SEONDAR! to $ree#isting disorders such as!enign tumours $aget disease !one infarcts

$re*ious radiation.@HISTOLO?I' FEAT@RES 3osteo!lasticchondro!lastic

2!ro!lastic telangiectatic small cell and


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(ROSSL: big b#l$y tumors. gritty% gray white% often contain areas of

haemorrhage and &ysti& 'egeneration. fre1uentl( destro(s surrounding cortices and $roduce soft tiss#e masses. s(rea' e)tensi*ely in the me'#llary &anal

in2l% trating and re$lacing the marrosurrounding

the $ree#isting !one tra!eculae. infre1uentl( (enetrate e(i(hyseal (late orenter

the +oint.

gros into it along tendoligamentous


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/ISTOLO(: tumour cells *ar( in si7e and sha$e. fre1uentl( ha*e large h($erchromatic

nuclei. !i77are tumor giant cells and mitosesarecommon.

t#e formation of bone by t#e tumorcells isc#aracteristic.

neo$lastic !one / usuall( coarse lace%li&e

archi% tecture. %ma( also !e !roadsheets or

$rimiti*e tra!eculae.


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&ascular in&asion /% usuall( cons$icious.

necrosis% % ma( !e $resent in536 to >36of indi*idual

tumour.


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Osteosarcoma+ A--ass in*ol*ing the

u$$er end of the ti!ia.

The tan%hite tumor 2llsmost of the medullar(

ca*it( of the meta$h(sisand $ro#imal dia$h(sis.

It has in2ltrated throughthe corte# lifted the

$eriosteum and formedsoft tissue masses on!oth sides of the !one


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B-/istologica))earance-

ith coarse laceli&e

$attern of neo$lastic!one 3arrow4 $roduced !(ana$lastic tumor cells.

Note the ildl( a!errantmitotic 2gures3arrowhea's,

CLINICAL COURSE


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CLINICAL COURSEt")ical )resentation / $ainful$rogressi*el( en%

largingmasses.sudden fractureof the !one% 3sometimes isthe

2rst s(m$tom4.

RA1IOLO(:large destructi&e mixed lytic andblastic mass 'it#

inltrati&e margins.the tumor !rea&s through the corte# and liftsthe $eriosteum resulting in reacti*e $eriosteal!one


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OSTEOSARCOMA

a tumor -T,in thefemoral dia$h(sisith a Co!mantriangle

Co!man triangle &hara&teristi& b#t


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Co!man triangle / &hara&teristi&% b#tnot

'iagnosti& of this

t#mor.SPREA1%mainl( aematogenousat the time of diagnosis 836 to 236ha*e

(#lmonarymetastases.in those ho die of this neo$lasm 36ha*e metastases to l#ngs% bones%

brain etc.

TREATMENT/ m#ltimo'ality a$$roach


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CARTILA(E ? FORMIN( TUMOU

tilage tumors account for the ma)orit(ofr( !one tumors and are c#aracteried byormation of #yaline or myxoid cartilage.

igncartilage tumors are much more common

malignant ones.

OSTEOC/ON1ROMA


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OSTEOC/ON1ROMA Benigncartilage *capped tumor that

is attached

to the underl(ing s&eleton !( a bonystalk. %also &non as E+,S-,SS

%most common benign bone tumora!out 56 are solitary./0-E ERE3-4R5 E+,S-,SSS563R,/E

autosomal dominant hereditar( disease.caused !( germline loss%of%functionmutations in either the ext1 or ext2genes+

inacti*ation of onl(ext1 has !een


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E% SOLITARY% usuall( 2rst diagnosed in latea'oles&en&e an' early a'#lthoo'.

-@LTIPLE3hereditar(4% !ecome a$$arentduring &hil'hoo'.

% men # >0 omen.E% de*elo$ onl( in !ones ofendochondral

origin and arise from the meta(hysis near the growth (late of long t#b#lar bones es$eciall( a!out the &nee. OASIONA!/ !ones of sca$ula $el*isand ri!s.% in these sites% sessile short stal&s. RARE! / short tu!ular !ones of the hands and feet.


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RP/OLO(:ssile or musroomsa)e!+

% range from 8 to 23cm+

nign "aline cartilage ca) *ar(ing in thic&ness.

$ is co*ered $eri$herall( !( )ericon!rium+

corte# of the stal& merges ith the corte# ofe host !one so that medullar( ca*it( of theteochondroma and !one are in continuit(.


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OSTEOC/ON1ROMA


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OSTEOC/ON1ROMA

CLINICAL FEATURES


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CLINICAL FEATURES $resent as slo groing masses. $ainful if im$inge on a ner*e or if the stal&

isfractured.

man( cases% incidental 2nding.

hereditar( e#ostosis / underl(ing !ones ma(!e!oed and shortened.

osteochondromas sto$$ed groingat timeof

groth $late closure.


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C/ON1ROMAS

Benign tumors of #yaline cartilage t#atusually

occurs in bones of enc#ondral origin.

if arise ithin the me!ullar" ca&it" / &nonas

E67,63R,/4S+

if arise on the surface of bone% &non as S0B8ER,S-E4 or90+-47,R-747,63R,/4S

3i i ll i 23 3 3 4


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3iagnosis/ usuall( in 23sto 3s+ 3(ears4.resentith solitar( meta$h(seal lesions oftu!ular !ones.

fa*ored sites% short tu!ular !ones of hands and feet.S(ndrome of multi)le encon!romas or

encon!romatosis is &non asOLLIER1ISEASE

Encon!romatosis associate! 'it soft%tissue

aemangiomas is called MAFFUCCIS:N1ROME

MORP/OLO(:% /


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/ISTOLO(:/ com$osed of ell%circumscri!ednodulesof c(tologicall( !enign h(alinecartilage. Peri$heral $ortions of nodules ma(

undergo enchondral ossi2cation and centercan

calcif( and die.In Ollier disease and -a,ucci s(ndrome /chondromas are more cellular and e#hi!itc(tologic at($ia.

CLINICAL FEATURES-ost enchondromas are as(mtomatic andare detected incidentall(.Occasionall( $ainful.

Encon!romatosis / tumors ma( !e numerous


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Encon!romatosis tumors ma( !e numerousand large $roducing se*ere deformities.

RA1IO(RAP/:

@nminerali7ed nodules of cartilage $roduce ell% circumscri!ed o*al lucencies that are

surrounded !( a thin rim of radiodense !one. ;7 or , ring signif matri# calci2es / irregular o$acities de*elo$.tumor nodules scallo$ the endosteum !ut

lea*e the corte# intact.

TREATMENT%+O /clinical situation. usuall( o!ser*ationor curettageencon!romatosis% Ris& of malignant

change.


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AFFUCCI S:N1ROME / ris& of de*elo$ing othermalignancies including o*arian &ar&inomas

and brain gliomas.


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ENC/ON1ROMA


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C/ON1ROMA

C/ON1ROBLASTOMA


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C/ON1ROBLASTOMA rare benign tumorC 7 86 of $rimar( !onetumors

A(E / usuall( (oung $atient in their teens SE % male" female 5 2.8 SITE % most / a!out the$nee.

in older $atients /(el*is an' ribs.

stri&ing $redelection for epip#ysesandapop#yses.

MORP/OLO(: com$osed of sheetsof com$act $ol(hedralchon% dro!lasts that ha*e ell%de2ned c(to$lasmic

!orders moderate amounts of $in& c(to$lasm

and nucleithat are h($erlo!ulatedith longi%

mour cells are surrounded !( scant #yaline


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mour cells are surrounded !( scant #yalinetrix- de$osited in a lace%li&e con2guration.

hen matri# calci2es it $roduces a character%

ticc#icken8'ire$attern of minerali7ation.attered non%neo$lastic osteoclast8type giantells$resent.ccasionall( undergo haemorrhagic c(stic

egeneration.

INICAL FEATURES

suall( $ainful.int e,usions.stricted )oint mo!ilit(.


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A1IO(RAP/:

ell%de2nedgeogra$hic lucenc( that commonl(has s$ott( calci2cations.

ecurrences/ not uncommon after curettage

arel(% $ulmonar(3 haemotogenous4metastases occur.


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C/ON1ROBLASTOMA


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Con!roblast

oma+ There is alarge destructi*ecalci2ed lesion ofthe $ro#imalhumeral e$i$h(sis

C/ON1ROM:OI1 FIBROMA


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C/ON1ROM:OI1 FIBROMArarestof cartilage tumors.can !e mista&en for sarcoma.

A(E % teensand 23s+ male$re$onderance.SITE% can in*ol*e an( !one of the !od(C most fre1entl( arise in the meta(hysisof

long t#b#lar bones.

(ROSS


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" C $ $aremost cellular.in cartilaginous regions / tumor cells aresituated in lacunaeCin m"#oi! areas / cells are stellate.*ar(ing degrees of c(tologic at($ia.

small foci of calci2cation.scattered non%neo$lastic osteoclast%t($egiant cells.

CLINICAL FEATURES / lo&ali/e' '#ll a&hy(ain.RA1IO(RAP/:/ eccentric geogra$hicl#&en&ythatis ell delineated from the ad)acent !one !(

a


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EATMENTof choice / sim)le curettage+ The( ma( recur !ut no threat for malignant

tranformation or metastasis.



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C/ON1ROSARCOMA


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C/ON1ROSARCOMA?rou$ of tumors that s$an a !road s$ectrumof

clinical and $athologic 2ndings.'ommon feature to all is% production of neoplastic cartilageS@B'LASSIFIE+according to"%SITE % central 3intramedullar(4

perip#eral3)u#tacortical andsurface4

HISTOLO?Y/ con&entional3h(aline andorm(#oid4 0clear cell

%dedi%erentiated

the second most common malignant matri#


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the second most common malignant matri# $roducing tumor of !one. A(E% clear cell and mesench(mal *ariants"%

occur in teensor 23s others / usuall( 3sor older.% male #20 female)856 of con*entional 3$eri$heral4chondro%

sarcomas arise from a $ree#isting enchondroma or osteochondroma

SITE/ commonl( arise in the central)ortions of te s,eleton including(el*issho#l'er and ribs.

The clear cell &ariant originates in

MORP/OLO(:


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MORP/OLO(:

(ROSS/ large bulky tumors: made up ofnodules of gray8'#ite translucentglistening tissue.

in m"#oi! &ariants the tumors are *iscousand gelatinousand the matri# oo7es from the cut

surface.s$ott( calci2cations% t($icall( $resent.central necrosiscreate c(stic s$aces.ad)acent corte#is thic&ened or eroded.

tumor gros ith !road $ushing fronts into

MICROSCOPICALL: ?


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C OSCO CCONGENTIONAL(RA1E 8 ;LOH (RA1E / mild

h($ercellularit(.'hondroc(tes / $lum$ *esicular nuclei ithsmall nucleoli.

Binucleate cells / s$arseC mitotic 2gures% fePortions of matri# fre1uentl( minerali7eC'artilage ma( undergo endochondralossi2cation.

(RA1E < % mar&ed h($ercellularit(D e#treme $leomor$hism ith!i77arre

tumor giant cells and mitoses.

CLEAR CELL C/ON1ROSARCOMA%


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CLEAR CELL C/ON1ROSARCOMAsheets of largemalignant chondroc(tes ith abundant clear

cytoplasm numerous osteoclast8type giantcellsand intralesional reacti&e bone formation.

MESENC/:MAL C/ON1ROSARCOMA /islands of 'ell8di%erentiated #yaline cartilagesurrounded !(sheets of small round cells.

CLINICAL FEATURES / $ainful $rogressi*el(enlarging masses.RA1IOLO(:/ $rominent endosteal scallo$ing.

foci of 8occulent densities 3calci2cations4

PRO(NOSIS


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PRO(NOSIS ?-(RA1E9$&e "ear sur&i&al / gra!e 85 36- gra!e 25 86- gra!e E7-Sel( common.

in 56G >BR,/4S / !e&elo)e! from


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,SS>56G >BR,/4S !e&elo)e! fromal !efectsthat gro to 5 or >cm in si7e.re usuall( not detected until adolescence.

P/OLO(:consist of gra( to (ello%!ron cellular

ns containing $broblastsand macro)agestioc(tes4.blastsare fre1uentl( arranged in a

iform 3$inheel4 $attern.

oc(tes are either multinucleated giant cellslusters of foam( macro$hages.

RA1IOLO(ICALL:/ $roduce elongated


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shar$l(demarcated radiolucenciesthat are

surrounded!( a thin rim of sclerosis.

CLINICAL FEATURES>BR,0S 7,R-74 3E>E7-S /

as"mtomatic usuall(detected radiologicall( as inci!ental2nding.

-ost / limited groth $otentialand undergos)ontaneous resolution ithin se*eral

(ears

to !e re$laced !( normal cortical !one.


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Fibrous cortical!efect=

Nonossif"ing$broma.

7#aracteristic

storiform pattern ofspindle cellsinterspersed 'it#scatteredosteoclast8typegiant cells.

NON OSSIF:IN( FIBROMA


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NON%OSSIF:IN( FIBROMA

FIBROUS 1:SPLASIA


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FIBROUS 1:SPLASIA Benign tumor li&ened to a localied

de&elopmental arrest

All the components of normal bone are$resent !ut the( do not di%erentiate into theirmature structures.Lesions arise during s&eletal groth andde*elo$ment.

T/REE CLINICAL PATTERNS"%

;8 monostotic"% in*ol*ement of single!one. ;2 )ol"ostotic "% in*ol*ement of multi$le!ones

;


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geneduring em!r(ogenesis.

MONOSTOTIC FIBROUS1:SPLASIA436 of all cases.occurse1uall( in !o(s and girls.usuallyin earl( adolescence and often sto$s enlarging at time of groth $late closure.

femur ti!ia ri!s )a !ones cal*aria andhumerus are most commonly a,ected.fre@uentlyas(m$tomatic and usuall(

disco*ered

can cause mar&ed enlargement and distortionf !


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of !one.craniofacial s,eleton in&ol&ementse*ere

dis2gurementcan occur.monostotic dAs does not e&ol&e intopolyostotic dAs.

POL:OSTOTIC FIBROUS 1:SPLASIAHIT/OUTEN1OCRINE 1:SFUNCTION246 of all casesmanifests at a slightl( earlier age than the monostotic t($e.femur s&ull ti!ia humerus ri!s 2!ula

radius

craniofacial in&ol&ement / $resent in 536to8336


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to8336+se*ere sometimes cri$$ling deformities3she$herd%croo& deformit( of $ro#imal femur4

and s$ontaneousand often recurrentfractures.

POL:OSTOTIC FIBROUS 1:SPLASIA

HIT/ CAFJ%AU%LAIT S0IN PI(MENTATION AN1EN1OCRINOPAT/IES

&non as /ccune84lbrig#t syndrome.account for


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usuall( limited to same side of !od(.Dclassicall( large.dar& to caf%au%lait.ha*e irregular ser$iginous !orders. 3 coastline of maine4found $rimaril( on the nec& chest !ac&

shoulder and $el*ic region.

MORP/OLO(:ell circumscri!ed intramedullar(.*ar( greatl( insi7e larger lesions e#$and and

distort the !one.

tan%hitein color and gritt(.

MICROSCOPICcom$osed of cur*ilinear tra!eculae of o*en


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com$osed of cur*ilinear tra!eculae of o*en !one surrounded !( a moderatel( cellular

2!ro% !lastic $roliferation. sha$es of tra!eculaemimicchinese letters.

the !one lac&s $rominent osteo!lasticrimming.nodules of h(aline cartilage ith a$$earanceof

disorgani7ed groth $late are also $resent+;236c(stic degeneration haemorrhage andfoam(

macro ha es are common.


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%RA1IOLO(:


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RA1IOLO(:T($ical ground%glass a$$earance and ell%de2ned margins.

$ol(ostotic disease is fre1uentl( associatedith $rogressi*e disease.

%SEGERE S0ELETAL COMPLICATIONS /recurring fractures long%!one deformities$ersistent $ain

distortion of craniofacial !ones.

%MALI(NANT TRANSFORMATION / rarecom$lication

usuall( from $ol(ostotic disease.

Fibrous


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Fibrous!"s)lasia+

'ur*ed tra!eculae ofo*en !one arising in

a 2!rous tissue.

Note the a!sence ofosteo!lasts rimming

the !ones.

FIBROUS 1:SPLASIA


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FIBROUS 1:SPLASIA

FIBROUS 1:SPLASIA


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FIBROUS 1:SPLASIA


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The u$$er left leg ith

t($ical osseous changesconsistent ith $brous!"s)lasia and se)er!Kscroo, !eformit"

FIBROSARCOMA GARIANTS


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ma( occur at an( age.mostl( middle%ageand elderl(.

nearl( e1ual se# distri!ution.usuall( arise de no*o !ut fe de*elo$ in $re%e#isting !enign tumors !one infarcts Pagetic!one and irradiated tissue.

MORP/OLO(:(ROSSL:% large haemorrhagic tan%hitemasses that destro(the underl(ing!one and

fre1uentl( e#tendsinto soft tissues.MICROSCOPIC% c(tologicall( malignant2!ro!lastsarranged in a herring!one storiform $attern.

FIBROSARCOMA-alignant s$indl( neo$lastic cells forming


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-alignant s$indl( neo$lastic cells forming/ERRIN(%BONE- STORIFORM$attern.

ICAL FEATURES ?


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rging $ainful mass.ll( arises in meta$h(sis of long !ones and

ic 8at !ones.ologic fracture is a fre1uent com$lication.

IO(RAP/: ? it is(ermeati*eandlyti&and oftdsinto the ad)acent soft tissue.

(NOSIS ?en!son / si/e lo&ation stage and gra'e of tumor.

MISCELLANEOUS TUMORSEHIN( SARCOMA9PRIMITIGE


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EHIN( SARCOMA9PRIMITIGENEUROECTO1ERMAL

TUMOR ;PNET$rimar( malignant small round8celltumors

of !one and soft tissue.share an identical chromosome translocation.to *ariants of the same tumor that di,er onl( in their degree of neural di,erentiation.

PNETs/ demonstrate neuraldi%erentiation.

EHIN( SARCOMA / undi%erentiated.

cond most common !one sarcomas in children.ungest a*erage age at $resentation%


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ungest a*erage age at $resentation%ost/ 83 to85"rs old- 36% (ounger than 23(rs

(s slightl(0 girlsi&ing $redilection for hitesC !lac&s are rarel( aist ha*e atranslocation in*ol*ing theSgeneon C/ROMOSOME 22 and a gene

co!ing an ETS famil" transcri)tion factor=ost common E-S geneis>11.

RP/OLO(:ft tan%hite and fre1uentl( contains areas of

morrhageand necrosis.risesin the medullar( ca*it(C usuall( in&a!estherte) (erioste#mand soft tiss#e.

ROSCOPICALL:/ com$osed of sheets of unifol ro#n' &ells slightl( larger than


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l ro#n' &ells slightl( larger thanhoc(tes. ith scant( clear c(to$lasm 3due to

gen4ER%HRI(/T rosettes3t#mor &ells arrange' in at a &entral 1brillary s(a&e4 is indicati*e of neuralrentiation.

or contains little stroma.rosis ma( !e $rominent.ti*el( fe mitotic 2gures.

ICAL FEATURESall( arise in dia$h(sisof long tu!ular !oneseciall( femurand 8at !ones of $el*is.ent as $ainful enlarging masses.

cted site is tender arm and sollen


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cted site is tender arm and sollen.temic $n!ings / fe*er ele*ated ESR anaemia&oc(tosis ma( !e $resent.

1IO(RAP/:% destructi*e l(tic tumor $ermeati*e margins

e#tension into surrounding soft tisiosteal reaction $roduces la(ers of reacti*ene in onion8skinfashion.

ATMENT/ chemothera$h( surgical e#cision ith or ithout irradiation.

(NOSIS/ a$$ro#imatel( 456 . 5%"rsur*i*alC at least 536 ha*e long%term cures.


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E'ing

sarcoma+

Sheets of small roundcells ith scant clearedc(to$lasm.

EHIN( SARCOMA


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EHIN( SARCOMA

EHIN( SARCOMA


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EHIN( SARCOMA

(IANT%CELL TUMOR


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containsa mi#ture of mononuclear cells and a$rofusion of multinucleated osteoclast%t($e

giant cells. s(non(m / osteoclastomarelati*el( uncommon.benign !ut locall" aggressi&e neo$lasm.age/ 23"rs to 3"rs+the mononuclear cells in the tumor e#$ress

R46C.3 Re&e(tor a&ti*ator of n#&lear fa&tor$a((a02 ligan'

also $nown as osteo&last 'i3erentiationfa&tor,

MORP/OLO(:(ROSS% large red%!ron tumors that fre1uentl(

undergo c(stic degeneration.MICROSCOPIC% com$osed mostl( of uniform o*al

mononuclear cells that

scatteredithin this !ac&ground are numerous osteoclast%t($e giant cells ha*ing833 or more


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nuclei that resem!le those of the mononuclear cells.

necrosis haemorrhage haemosiderinde$osition and reacti*e !one formation are common.

CLINICAL FEATURESA1ULTS % in*ol*e !oth thee$i$h(ses and themeta$h(ses.

A1OLESCENTS% con2ned $ro#imall( !( thegroth $late and are limited tometa$h(sisMAORIT:% arise around the &nee3 distal femurand

$ro#imal ti!ia4.

fre1uentl( causes arthritis%li&e s(m$toms.


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1 ( ( $occasionall( / $athologic fractures.most are solitar(C

multi$lemulticentric tumors occur es$eciall( in the e#tremities.often erode into su!chondral !one $lateC destro( o*erl(ing corte#C $roduce !ulging soft

tissue massdelineated !( a thin shell of reacti*e !one.

TREATMENT

curettage/ associated ith a 36 to >36recurrence rate.u$ to 6 metastasi7e to the lungs.


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Benign giant%cell

tumor shoing a!undant

multinucleated giant cells

and a !ac&ground ofmononuclear cells.

(IANT CELL TUMOR OF BONE


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ANEUR:SMAL BONE C:STA benign tumor of !one characteri7ed !( multi


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A benign tumor of !one characteri7ed !( multi8 loculated blood8lled cystic spacesthat ma( $resent as a ra$idl( groing e#$ansile tumor. associated ith distincti*e 84)8 adeu!i1uitinating

en7(me.MORP/OLO(:(ROSS% multiple blood8lled cystic spacesseperated

by t#in$ tan8'#ite septa.Microsco)icall"Hallsare com$osed of $lum$ uniform 2!ro!lastsmultinucleated osteoclast%li&e giant cells and

reacti*e o*en !one.

a$$ro#imatel( one tir! of cases contain acartilage%li&e matri#called blue bone+


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necrosis is uncommon.

CLINICAL FEATURESa,ects all age grou$s.generall( occurs during $rst 2 !eca!es of life.

no se# $redilection.

most fre1uentl( de*elo$s in meta$h(ses of long!onesand the $osterior elements of *erte!ral

!odies.most common s9s % )ainand s'elling+

&ertebral in&ol&ementcom$ress ner*es neurologic s(m$toms.

rel( $athologic fractures occur.


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( $ g

1IO(RAP/:/ aneccentric e)(ansilelesion ithl% de2ned margins. -ost lesions are com$letel(cand contain a thin shell of reacti*e !one at$eri$her(andMRIma( demonstrate internal se$ta and

aracteristic 8uid le*els.

EATMENT% surgical C curettage or en !loc resection

currence rate is loContaneous regression ma( occur folloingcom$lete remo*al.

ANEUR:SMAL BONE C:ST


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-@LTIPLE BLOO+%FILLE+ 'YSTI' SPA'ESSEPERATE+ BY THINFIBRO@S SEPTA

ANEUR:SMAL BONE C:ST


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METASTATIC TUMORS


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most common s&eletal malignanc(.usuall( occur in late stages of tumor.

PAT/HA:Sof s$read include" ;.3RE7- EGTENSION . 5/4-7OR 4E/4-,GE6,0S+ISSE-INATION >. 6-R4S64 SEE+LIN? 3*ia Batson$le#us of *eins4

INADUTS" more than 456 originate from"% cancers of prostate- breast- kidneyandlung.

IN 4IDREN" originate from"%

icall( multifocal 3e#ce$t&idne( and th(roidduce solitar( lesions4


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duce solitar( lesions4.( occur in any boneCmostin*ol*e0 a)ial s$eleton-*ertebrall#mn% (el*is% ribs% s$#ll% stern#m,% (ro)imal

#raand h#mer#s in descending order.tastasis to small bones of han' an' feet areommonand usuall( from cancer of l#ng

eyand &olon.

IO(RAP/:%( !e $urel(L:TIC $urel( BLASTIC or MIE1

TIC AN1 BLASTIC.cinoma of kidney$ lung$ G.. tract$ malignantelanoma$ producelyticlesions+

prostatea!enocarcinoma%elicit scleroticres$onse.


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res$onse.most oter metastases induce ami#e!l(ticand

!lastic reaction inl"ticlesions/ metastatic cells secrete

su!stances such as"prostaglandins-cytokines and-8relatedproteinthat stimulate osteoclastic !one resor$% tionC the tumor cells themsel*es do not directl( resor! !one.

l"tic bone tissue /0 rich in groth factors*i7. T(F%b- I(F%i- F(F- P1(F and bonemor)ogenetic

)roteins% hel$ create an en*ironment


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Radiogra$h shosa dis$laced

fracture through anosteolytic lesionin the distal femurof a =>%(ear%old

oman ith lungcarcinoma


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Pel*ic radiogra$h

shos ides$readosteoblastic$scleroticmetastases from

$rostate cancer

!NC!"ENCE O# BONE C$NCER


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%&O bone sarcoma account for '()* of all

neoplasms. (2006)

!"#$ %&'"% appro*imately'(+ ne+ cases

per ,00-000 population and

year

%rgentina and ra/il higher incidence

'1% (34) (2005) &%1' , -() . /''0''' 7'&%1'1 2(' . /''0'''

O# $LL M$L!GN$NT 3R!M$R4 BONE TUMORS 1

osteosarcoma , 25* chondrosarcoma1 )5*

e+ing sarcoma /6*

fibrosarcoma , 5*

( S''" 8rogramme ,9:;


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BONE C$NCER !N M$L$4S!$

N$T!ON$L C$NCER REG!STR4 7M$L$4S!$8C$NCER !NC!"ENCE !N M$L$4S!$ 7)''29)''58

M$LE 1 22' C$SES: /(/ . /''0'''

#EM$LE 1 )'' C$SES: '(5 . /''0'''


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THAN9 YO@

BONE TUMOURS - I & II - 2015.pptx

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