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    Bone Tumors OverviewFor Medical Students

    Abdelfattah Fawzi Khdeir M.D.

    Ortho & Trauma esident!ebron " #$%

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    A classification of bone tumors. Modified after evised '!O (lassification ) Scha*owicz +%,,-

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    !STO/

    As0m1tomatic until the abnormalit0 is discovered on 2"ra0.

     Must be ca1able of s1ontaneous resolution

    Situated where there is room for incons1icuous e21ansion

     Age :

    Durin3 childhood """ +fourth or si2th decades-

    M0eloma is seldom seen before the si2th decade

    In patients > 70 years of age, metastatic bone lesions are more

    common than all primary tumors together.

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    !STO/

    4ain 5

    a1id e21ansion with stretchin3 of surroundin3

    tissues

    (entral haemorrha3e

    De3eneration in the tumour

    4atholo3ical fracture

    6er0 1ainful if it is enca1sulated in dense bone

    +e.3. an osteoid osteoma-.

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    !STO/

    • Neurological symptoms (paraesthesiae or

    numbness)

    • Pathological fracture may be the first (an only)

    clinical si3nal.

    n elderl0 1eo1le an0 brea7 in the mid"shaftshould be re3arded as 1atholo3ical until 1roved

    otherwise.

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    89AM:ATO:

    • A lump , !here oes it arise" Is it iscrete or ill"defined; s it soft or

    hard< or 1ulsatile; And is it tender;

    • #!elling is sometimes iffuse, an the o$erlying s7in warm and

    inflamed= it can be difficult to distin3uish a tumor from infection or ahaematoma.

    • f the tumor is near a *oint there ma0 be an effusion and>or limitation of

    mo$ement.

    • #pinal lesions , whether beni3n or mali3nant< often cause muscle

    spasm an bac% stiffness, or a painful scoliosis

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    9"A/S

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    9"A/S

    • (ortical thic7enin3< a discrete lum1< a ?c0st@ or ill"defined destruction.

    • 'here is the lesion5 in the meta1h0sis or the dia1h0sis;

    • s it solitar0 or are there multi1le lesions;

    • Are the mar3ins well"defined or ill"defined;

    • emember that ?c0stic@ lesions are not necessaril0 hollow cavities5 an0

    radiolucent material +e.3. a fibroma or a chondroma- ma0 loo7 li7e a

    c0st.

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    9"A/S

    • f the boundar0 of the ?c0st@ is shar1l0 defined it is 1robabl0 beni3n= if

    it is haz0 and diffuse it su33ests an invasive tumour.

    • Sti11led calcification inside a c0stic area is characteristic of cartila3e

    tumors.

    • oo7 carefull0 at the bone surfaces5 1eriosteal newbone formation and

    e2tension of the tumour into the soft tissues are su33estive of

    mali3nant chan3e.

    • oo7 also at the soft tissues5 Are the muscle 1lanes distorted b0

    swellin3; s there calcification;

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    ADO:(D8 S(A:::C

    • Scannin3 with ,,mTc"meth0l di1hos1honate +,,mTc"

    MD4- shows non"s1ecific reactive chan3es in bone

    • This can be hel1ful in revealin3 the site of a small

    tumour +e.3. an osteoid osteoma- that does not show

    u1 clearl0 on 2"ra0.

    • s also useful for detectin3 s7i1 lesions or ?silent@

    secondar0 de1osits.

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    (OM4T8D TOMOCA4!/

    • t shows more accuratel0 both intraosseous and

    e2traosseous e2tension

    • eveal sus1ected lesions in inaccessible sites<

    li7e the s1ine or 1elvis

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    M

    • ts 3reatest value is in the assessment of tumor

    s1read5 +a- within the bone< +b- into a nearb0 *oint

    and +c- into the soft tissues.

    • Blood vessels and the relationshi1 of the tumor to

    the 1erivascular s1ace are well defined.

    • M is also useful in assessin3 soft"tissue tumours

    and cartila3inous lesions.

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    • Tumors ) differential dia3nosis

    +a- This hu3e swellin3 was sim1l0 a clotted haematoma.

    +b- Bone infection with 1atholo3ical fracture.

    +c- Florid callus in an un"united fracture.

    +d- ar3e erosion in the calcaneum b0 a 3out0 to1hus.

    +e- Bone infarcts

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    STAC:C OF BO:8 TMOS

    • # s0stems " one for mali3nant lesions and one for beni3n lesions

     – Mali3nant lesions are defined usin3 oman numerals +e.3. < < -

    • e.3. osteosarcoma most commonl0 1resents as sta3e B

     – Beni3n lesions are defined usin3 Arabic numbers +%

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    STAC:C OF BO:8 TMOS

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    STAC:C OF BO:8 TMOS

    • Tumor (om1artments

     – ntracom1artmental• bone tumors are confined within the corte2 of the bone

     –

    82tracom1artmental• bone tumors e2tend be0ond the bone corte2

    • Tumor Crade

     – !istolo3icall0< tumors are 3raded based on the 1ercenta3e of cellular at01ia • ow 3rade tumors

     – low metastatic 1otential

     – e.3. 1arosteal osteosarcoma 

    • !i3h 3rade tumors – 3reater metastastatic 1otential

     – e.3. intramedullar0 osteosarcoma< 8win3s sarcoma< dedifferentiated chondrosarcoma 

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    MSTS +8nne7in3- Sta3in3 S0stem

    • Musculoskeletal Tumor Society system

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    • A 13-year-old girl presents with an isolated distal femur

    osteosarcoma that extends into the soft tissue. Work-up is negatie

    for metastasis! "ut "iopsy reeals a high grade lesion. What is the

    stage of this tumor "y the Musculoskeletal Tumor Society system#

    1. $ A

    • %. $$ A

    3. $ &• '. $$ &

    • (. $$$

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    •  +a- 4lain 2"ra0 shows a destructive lesion of the 1ro2imal tibia< almost certainl0 an osteosarcoma 

    •  +b

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    B8:C: BO:8 8SO:S

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    :O:"OSSF/:C FBOMA +FBOS

    (OT(A D8F8(T-

    • The commonest beni3n lesion of bone.

    • A develo1mental defect in which a nest of fibrous tissue

    a11ears within the bone and 1ersists for some 0earsbefore ossif0in3.

    • t is as0m1tomatic and is almost alwa0s encountered in

    children as an incidental findin3 on 2"ra0.

    • The commonest sites are the meta1h0ses of lon3 bones

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    :O:"OSSF/:C FBOMA +FBOS

    (OT(A D8F8(T-

    • (ommon in children G"%G 0ears old

    +8stimated that $H of children with o1en 1h0sis

    have a nonossif0in3 fibroma-

    • I$H in lower e2tremit0

    • (ommon locations include the 7nee +distal femur and

    1ro2imal tibia- and distal tibia 

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    :O:"OSSF/:C FBOMA +FBOS

    (OT(A D8F8(T-

    • The &'ray appearance is meta1h0seal eccentric Jbubbl0J

    l0tic lesion surrounded b0 sclerotic rim

    • views in different 1lanes ma0 show that a lesion thata11ears to be ?central@ is actuall0 ad*acent to or within the

    corte2< hence the alternative name ?fibrous cortical defect@.

    •  Although it loo%s cystic on &'ray, it is a solid lesionconsistin3 of unremar7able fibrous tissue with a few

    scattered 3iant cells.

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    :O:"OSSF/:C FBOMA +FBOS

    (OT(A D8F8(T-

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    :O:"OSSF/:C FBOMA +FBOS

    (OT(A D8F8(T-

    • As the bone 3rows the defect becomes less obvious and it

    eventuall0 heals s1ontaneousl0.

    • There is no ris7 of mali3nant chan3e.

    • reatment is usually unnecessary.

    •  If thedefect is ver0 lar3e or has led to re1eated fractures< it can betreated b0 curetta3e and bone 3raftin3.

    • ecurrence is rare.

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    FBOS D/S4ASA

    • Areas of trabecular bone are re1laced b0

    cellular fibrous tissue containin3 flec7s of

    osteoid and woven bone.

    • t ma0 affect 5

     One bone +monostotic-

     One limb+monomelic-

     Man0 bones +1ol0ostotic-.

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    FBOS D/S4ASA

    Females males

    Found in an0 and all a3es

    Onset for LGH of 1atients at $ 0ears of a3e

    An0 bone can be involved

    The 1ro2imal femur is most common site

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    Mc(une Albri3ht s0ndrome

    (ondition defined b0 5

    The 1resence of s7in abnormalities +cafe au lait s1ots-

    8ndocrine abnormalities 5

    +4recocious 1ubert0-

    enal 1hos1hate wasin3 due to FCF"# +onco3enic

    osteomalacia-

    4ol0ostotic fibrous d0s1lasia 

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    +(afe au lait s1ots-

     ar3er and more irre3ular borders than neurofibromatosis

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    4ol0ostotic Fibrous D0s1lasia 

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    N4unched"outJ lesion with well defined mar3in of sclerotic bone

    !i3hl0 l0tic lesions or a 3round 3lass a11earance

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    ?She1herd@s (roo7@ Deformit0

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    Treatment

    • :ono1erative

     – observation

    • indications

     – as0m1tomatic 1atients

     – Di1hos1honate thera10

    • indications – s0m1tomatic 1ol0ostotic fibrous d0s1lasia 

    • effective in decreasein3 1ain and reducin3 bone turnover

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    Treatment

    • O1erative

     – internal fi2ation and bone 3raftin3

    • indications – s0m1tomatic lesions

     –

    im1endin3>actual fractures throu3h lesions in areas of hi3h stress +femoral nec7- – severe deformit0

     – neurolo3ic com1romise in the s1ine

    • techniue – never use auto3enous cancellous bone< as the trans1lanted bone will uic7l0 turn into

    fibrous d0s1lastic woven bone

     – use cortical or cancellous allo3rafts – intramedullar0 device more effective than a 1late in the lower e2tremit0

     – Osteotomies for deformities

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    • Mali3nant transformation to fibrosarcoma

    occurs in $.G 1er cent of 1atients with

    monostotic lesions and u1 to G 1er cent of

    1atients with Albri3ht@s s0ndrome.

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    OST8OD OST8OMA

    • This tin0 bone tumour + %.G cm in diameter- causes

    s0m1toms out of all 1ro1ortion to its size.

    • 4atients are usuall0 under $ 0ears of a3e and males1redominate.

    • An0 bone e2ce1t the s7ull ma0 be affected< but over half

    the cases occur in the femur or tibia.

    • T01icall0 the 1ain is relieved b0 salic0lates.

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    OST8OD OST8OMA

    • G$H in dia1h0sis or meta1h0sis of lon3 bones of lower

    e2tremit0 +tibia< femur-

     – 1ro2imal femur tibia dia1h0sis 1osterior elements of the s1ine

    fin3ers and car1us feet

    • The most common location is the 1ro2imal femur

    • The most common intra"articular location is the hi1 *oint

    • The most common locations in the hand are the sca1hoid and

    1ro2imal 1halan2

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    OST8OD OST8OMA

    • 4atho1h0siolo30

     – thou3ht to be from nerve fibers associated with blood vessels within the nidus

     – 1ain is secondar0 to 1rosta3landin secretion and (O9%># e21ression

    • (O9%># e21ression in tumor ma7es it sensitive to :SAD thera10

    • Associated conditions

     – ortho1aedic manifestations

    • 1ainful scoliosis

    • 3rowth disturbance

    • fle2ion contractures

    • 4ro3nosis

     – 1ain from lesions usuall0 resolves after an avera3e of 0ears

     – the lesion s1ontaneousl0 resolves in G"L 0ears

     – in the s1ine< earl0 resection +within %I months- leads to resolution of scoliosis in

    0oun3 children +%%0ears-

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    OST8OD OST8OMA

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    OST8OD OST8OMA

    • S0m1toms

     – 1ain that

    • increases with time

    •  worse at ni3ht and with drin7in3 8TO!• relieved b0 :SADS 

    • ma0 be ad*acent to *oint and mimic arthritis

     – hand lesions ma0 1resent with 1ainless swellin3

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    OST8OD OST8OMA

    • 4h0sical e2am

     – Point effusions

     – (ontractures

     – im1

     – Muscle atro1h0

     – Ma0 1resent as 1ainful nonstructural scoliosis

    • As a result of 1aravertebral s1asm• The osteoid osteoma is located on the concave side at

    the a1e2 of the curve

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    OST8OD OST8OMA

    • adio3ra1hs

     – ntensl0 reactive bone around radiolucent nidus

    • :idus is %.G cm +otherwise osteoblastoma-

    • :idus ma0 be difficult to see on 1lain 2ra0 – Because intense 1eriosteal reaction ma0 obscure the nidus

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    OST8OD OST8OMA

    • The im1ortant &'ray feature is a small raiolucent area< the so"called ?nidus@.

    •  esions in the dia1h0sis are surrounded b0 dense sclerosis and cortical

    thic7enin3= this ma0 be so mar7ed that the nidus can be seen onl0 in fine

    cut (T scans.

    • esions in the meta1h0sis show less cortical thic7enin3.

    • ,,mTc"MD4 scinti3ra1h0 reveals intense< localized activit0.

    • t is sometimes difficult to distin3uish from a small Brodie@s abscess without

    bio1s0.

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    Osteoid osteoma The 2"ra0 a11earance de1ends on the site of the lesion.

    +a- 'ith cortical tumours there is mar7ed reactive bone thic7enin3 leavin3 a small lucent nidus

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    OST8OD OST8OMA

    • (T 5

    • Stud0 of choice

    • To identif0 nidus surrounded b0 a sclerotic rim

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    OST8OD OST8OMA

    • Bone scan 5

    • alwa0s hot with intense focal u1ta7e

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    Treatment

    • :ono1erative

     – clinical observation and :SAD administration

    • indications

     – :SADS are %st line and will lead to a dramatic decrease ins0m1toms

    » QG$H can be treated with :SADS alone

     – Also indicated for 1ainful s1ine lesions without scoliosis

     – Fin3erti1 lesions +distal 1halan2- ma0 not res1ond to :SADS

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    OST8OD OST8OMA

    • 4ercutaneous radiofreuenc0 ablation 

     – elative indications

    • Failure of medical mana3ement

    • 4eriarticular lesions< which increase the ris7 of cartila3e in*ur0 and 1remature de3enerative disease.

    • S1inal lesions +controversial- " de1ends on the location of the lesion and 1ro2imit0 to neural elements

     – (ontraindications

    • lesions close to s1inal cord or nerve roots

     – Techniue

    • done under (T 3uidance

    • 1robe at I$",$ de3 ( for R minutes to 1roduce a %cm zone of necrosis

     – Outcomes

    • ,$H of 1atients are successfull0 treated with %"# sessions of FA

    • %$"%GH recurrence rate

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    OST8OD OST8OMA

    • Sur3ical resection indications

     – ocation of lesion is not amenable to (T 3uided

    1ercutaneous radiofreuenc0 ablation

     – S1ine lesion associated with 1ainful scoliosis

     – f the e2cision is li7el0 to wea7en the host bone +es1eciall0

    in the vulnerable medial corte2 of the femoral nec7-<

    1ro1h0lactic internal fi2ation ma0 be needed.

    http://upload.orthobullets.com/topic/8012/images/osteoid%20osteoma%202.jpghttp://upload.orthobullets.com/topic/8012/images/osteoid%20osteoma%201.jpg

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    Osteoblastoma 

     +CA:T OST8OD OST8OMA-

    • A33ressive beni3n osteoblastic tumor of bone

     – Jbi3 brotherJ of osteoid osteoma +nidus #cm-

    • elativel0 rare

    • less common than osteoid osteoma 

    • males females +#5%-

    • ma*orit0 of 1atients %$"$ 0ears of a3e

     – location• most common in 1osterior elements of s1ine

    • Associated conditions

     – onco3enic osteomalacia 

     – secondar0 AB(

    • %$H"$H associated with secondar0 AB(

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    Osteoblastoma 

    • S0m1toms

     – 4ain

    • slowl0 1ro3ressive dull achin3 1ain

    • not relieved b0 :SADS – Ma0 see neurolo3ic s0m1toms with s1ine involvement

    • 4h0sical e2am

     – Swellin3

     – Muscle atro1h0

     – im1

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    Osteoblastoma 

    • adio3ra1hs

     – findin3s

    • l0tic or mi2ed l0tic"blastic lesion with radiolucent nidus #cm

    • reactive sclerotic bone

    • RRH corticall0 based< H medullar0 based• often e21ansile with e2tension into soft tissues with rim of reactive bone

     – #GH a11ear ver0 a33ressive and often mista7en for mali3nant lesion

    • (T

     – indications

    • necessar0 to full0 evaluate lesion

    • Bone scan

     – hot with intense focal u1ta7e

    http://upload.orthobullets.com/topic/8013/images/osteoblastoma%20ct.jpg

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    Osteoblastoma 

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    Osteoblastoma 

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    Osteoblastoma 

    • :ono1erative

     – observation

    • indications

     – rarel0< if ever< indicated as the lesion will continue to 3row

    • O1erative

     – curetta3e or mar3inal e2cision with bone 3raftin3

    • indications – standard of care

    • recurrence %$"#$H

    (hondroma +a- The hand is a common site

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    (hondroma +a- The hand is a common site.

    +b- Another chondroma before and after curetta3e and

    bone 3raftin3.

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    8nchondromas

    • location – usuall0 found in the medullar0 cavit0 of

    the dia1h0sis or meta1h0sis

     – the most common location is the hand +R$H-

    »

    the most common bone tumor in the hand is theenchondroma 

     – other locations include the distal femur +#$H-< 1ro2imal

    humerus +%$H- and tibia 

     – rare in the s1ine and 1elvis

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    8nchondromas

    • 4atho1h0siolo30 5

    • enchondromas re1resent incom1lete

    endochondral ossification

     – chondroblasts and fra3ments of e1i1h0seal

    cartila3e esca1e from the 1h0sis< dis1lace into the

    meta1h0sis and 1roliferate there

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    Olliers disease +Multi1e 8nchondromatosis-

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    8nchondromas

     – Maffuccis s0ndrome

    • S1oradic inheritance with no 3enetic 1redis1osition

    • Multi1le enchondromas and soft"tissue an3iomas 

    • adio3ra1hicall0< enchondromas in Maffuccis s0ndrome mar7edl0e21and the bone and an3iomas are seen as small< round calcified

    1hleboliths

    is7 of mali3nant transformation u1 to %$$H 

    • Also has increased ris7 of visceral mali3nancies +astroc0toma< C

    mali3nanc0-

    http://upload.orthobullets.com/topic/8018/images/maffucci%20foot.jpg

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    Maffuccis s0ndrome

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    Maffuccis s0ndrome

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    8nchondromas

    • S0m1toms

     – As0m1tomatic< discovered incidentall0 on

    radio3ra1hs

    • usuall0 true for enchondromas in lon3 bones and foot – 4atholo3ic fracture

    • often seen with enchondromas in the hand

    http://upload.orthobullets.com/topic/8018/images/patho%20fracture%202.jpg

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    8nchondromas

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    8nchondromas

     – 4ain• 4ain is uncommon

    'hen a 1atient 1resents with an enchondroma and 1ainin the ad*acent *oint< the cause of 1ain is often unrelated

    to the tumor

    • nli7e enchondroma< most chondrosarcomas have non"mechanical 1ain +rest 1ain and nocturnal 1ain-

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    8nchondromas

    • 4h0sical e2am

     – Shortenin3 and an3ular deformities• enchondromas ma0 disru1t the 3rowth 1late

     – Multi1le bluish an3iomas in Maffuccis s0ndrome

    http://upload.orthobullets.com/topic/8018/images/maffucci%20hand2.jpg

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    8nchondromas

    • adio3ra1hs• s7eletal surve0 if 1ol0ostotic disease is sus1ected

     – Findin3s

    • 'ell defined< lucent< central medullar0 lesions that calcif0over time

    • % to %$cm in size – meta1h0seal location when the0 first a11ear

     –

    a11ear more dia1h0seal as the lon3 bone 3rows• N4o1"cornJ sti11led calcification and rin3s

    • Minimal endosteal erosion +G$H width of corte2-

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    N4o1"cornJ sti11led calcification and rin3s

    " (ortical e21ansion and thinnin3 ma0 be 1resent +8s1eciall0 in the hand-

    http://upload.orthobullets.com/topic/8018/images/maffucci%20hand%20xr2.jpg

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    " Ma0 have 1urel0 l0tic a11earance +8s1eciall0 in hand-

    Olliers disease

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    Ollier s disease

    enchondromas mar7edl0 e21and the bone

    bones are d0s1lastic< with shortenin3 and bowin3

    Maffuccis s0ndrome

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    Maffucci s s0ndrome

    8nchondromas mar7edl0 e21and the bone

    An3iomas are visible as calcified 1hleboliths

    http://upload.orthobullets.com/topic/8018/images/maffucci%20hand%20xr1.jpghttp://upload.orthobullets.com/topic/8018/images/maffucci%20xr.jpg

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    8nchondromas

    • nli7e enchondromas< chondrosarcomas dis1la0 – (ortical thic7enin3 and destruction

     – 8ndosteal erosions and scallo1in3 G$H of the width of the

    corte2

     –

    Are lar3er +Gcm-

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    8nchondromas

    • :ono1erative

     – Observation

    • indications –

    Treatment for vast ma*orit0 of as0m1tomatic enchondromas• Follow u1

     – Serial radio3ra1hs for interval 3rowth +ever0 "R months for %"#

    0ears< then annuall0-

     – on3 term follow"u1 for 1atients with multi1le enchondroma

    s0ndromes

    h d

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    8nchondromas

    • O1erative – intralesional curetta3e and bone 3raftin3

    • indications – lesion that shows an0 chan3e on serial 2ra0s

     –

    radio3ra1hs sus1icious for low"3rade chondrosarcoma  – lar3e lesions at ris7 for recurrent fracture

    • outcomes – local recurrence is unusual

     – immobilization< followed b0 curretta3e and bone 3raftin3

    • indications – 1atholo3ic fracture in small tubular bones +hand lesions-

    • techniue – immobilize until fracture union< followed b0 curretta3e and 3raftin3

    h d

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    8nchondromas

    • Mali3nant transformation

     – is7 of transformation of enchondroma to low"3rade

    chondrosarcoma 

    • Solitar0 enchondroma – ris7 of transformation is %H

    • Olliers disease +multi1le enchondromatosis- – ris7 of transformation is #G"$H

    Maffuccis s0ndrome – ris7 of transformation is #"%$$H

     – also has hi3h ris7 of fatal visceral mali3nanc0

    (h d bl

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    (hondroblastoma 

    • A beni3n chondro3enic lesion +differs from 3iantcell tumor b0 its chondroid matri2-

    • #5% male5female

    • most 1atients under #G 0ears of a3e

     – ocation

    • 81i1h0seal lesion in 0oun3 1atients +usuall0 around %# 0ears

    of a3e-

    • (ommon locations include distal femur< 1ro2imal tibia<

    1ro2imal humerus< 1ro2imal femur< and a1o1h0sis or triradiate

    cartila3e of the 1elvis

    • T01icall0 e1i1h0seal but ma0 occasionall0 cross the 1h0sis

    (h d bl

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    (hondroblastoma 

    • 4atho1h0siolo30

     – Thou3ht to arise from cartila3inous e1i1h0seal 1late

    • 4ro3nosis

     – %"#H of beni3n chondroblasts metastasize to the

    lun3s +similar to 3iant cell tumor in this res1ect-

     – ecurrence is %$H"%GH after sur3ical resection

    (h d bl t

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    (hondroblastoma 

    • 'ell"circumscribed e1i1h0seal l0tic lesion with thin rim ofsclerotic bone that is shar1l0 demarcated from normal

    medullar0 cavit0

    • esions often cross 1h0sis into meta1h0sis

    • Sti11led calcifications within the lesion ma0 or ma0 not be

    1resent +#GH"GH-

    • (ortical e21ansion ma0 be 1resent

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    (h d bl t

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    (hondroblastoma 

    • O1erative – e2tended intralesional curetta3e and bone 3raftin3

    • indications –

    standard of treatment in s0m1tomatic individuals• techniue

     – ma0 do local ad*uvant treatment with 1henol or cr0othera10

     – esection of rare beni3n 1ulmonar0 metastasis

    • indications – if 1ulmonar0 metastasis

    + -

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    OST8O(!O:DOMA +(ATAC8"(A448D 89OSTOSS-

    • A beni3n chondro3enic lesion derived from aberrant cartila3e fromthe 1erichondral rin3 that ma0 ta7e the form of

     – Solitar0 ostoechondroma 

     – Multi1le !ereditar0 82ostosis +M!8-

    The most common beni3n bone tumor

    (ommon in adolescents and 0oun3 adults +teste ages: , *0, *+, +0, +-

    ocation

    • Occur on the surface of the bone and often at sites of tendon insertion

    (ommon locations include – 7nee +1ro2imal tibia< distal femur-

     – 1ro2imal femur

     – 1ro2imal humerus

     – subun3al e2ostosis +occurs most often at hallu2-

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    OST8O(!O:DOMA

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    OST8O(!O:DOMA

    • 4atho1h0siolo30 – Solitar0 osteochondromas can arise because of

    • Salter"!arris fracture

    • Sur3er0

    • adiation thera10 +commonest beni3n radiation"induced bonetumor-

     – 4athoanatom0

    • !amartomatous 1roliferation of bone and cartila3e

    • 4ossibl0 arise from 3rowth 1late cartila3e that 3rows throu3h thecorte2 b0 endochondral ossification under the 1eriosteum

    • Stal7 of the lesion is cortical and cancellous bone formed from

    ossified cartila3e

    OST8O(!O:DOMA

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    OST8O(!O:DOMA

    • Associated conditions – secondar0 chondrosarcoma

    • A mali3nant condition that results from mali3nant transformation of a

    solitar0 osteochondroma or M!8  " Occurs in older 1atients +tested a3es5 G$- – Most common location of secondar0 chondrosarcoma is the 1elvis +usuall0 occur as

    low"3rade chondrosarcomas-

    • 4ro3nosis

     –

    is7 of mali3nant transformation is

    • %H with solitar0 osteochondroma 

    • QG"%$H with M!8 develo1 secondar0 chondrosarcoma 

    M lti1le !ereditar 8 ostosis +M!8-

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    Multi1le !ereditar0 82ostosis +M!8-

    • Disorder characterized b0 multi1le osteochondromas

    • Other name Multi1le Dia1h0sial Aclasis

    • 4atho1h0siolo30

     –

    Mutations affect the 1reh01ertro1hic chondroc0tes of the 1h0sis• Autosomal dominant

    • 4ro3nosis

     – GH"%$H mali3nant transformation to chondrosarcoma in

    1atients with M!8

     – 4ro2imal lesions more li7el0 to under3o mali3nant

    transformation than distal lesions

    Osteochondroma

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    Osteochondroma 

     – S0m1toms

    • Most lesions are as0m1tomatic

    • suall0 1resent with 1ainless mass• Ma0 have mechanical s0m1toms or s0m1toms of

    neurovascular com1ression

    • The0 continue to 3row until s7eletal maturit0

     – 4h0sical e2am• 4al1able mass

    • ma0 have mechanical s0m1toms secondar0 to mass

    Multi1le hereditar0 e2ostosis +M!8-

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    1 0 + -

     – S0m1toms• imb deformities

     – Most common sites of deformit0 include the 7nee< forearm< and an7le

    » Femoral shortenin3 and limb"len3th discre1anc0

    » (o2a val3a 

    » Knee val3us +because of shortened fibula- and 1atellar dislocation

    » An7le val3us +because of shortened fibula-

     – 11er e2tremit0 deformities are well tolerated and lead to little loss of function

    » lnar shortenin3

    » adial bowin3 and radial head dislocation

    » Ma0 be treated with e2ostosis e2cision< ulnar len3thenin3 and radial closin3 wed3e

    osteotom0

    • Point 1ain – ma0 have s0m1toms of 1remature OA

    Multi1le hereditar0 e2ostosis +M!8-

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    1 0 + -

     – 4h0sical e2am• Most common deformities include

     – lnar shortenin3 and radial bowin3

     – adial head dislocation

     – lnar deviation of the hand

    • Secondar0 chondrosarcoma

     – acute onset of 1ain in adults with M!8 should raise

    sus1icion for mali3nanc0

    Osteochondroma

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    Osteochondroma 

    • adio3ra1h – Sessile +broad base- or 1edunculated +narrow stal7- lesions found on

    the surface of bones

    • hi3her ris7 of mali3nant de3eneration in sessile lesions

    • 1edunculated lesions 1oint awa0 from the *oint

     – (ontinuit0 with native tissue• corte2 of the lesion continuous with corte2 of the native bone

    • medullar0 cavit0 of lesion continuous with medullar0 cavit0 of native bone 

     – (artila3e ca1 is usuall0 radiolucent and involutes at s7eletal maturit0

     – :odules of meta1lastic cartila3e can occur within the bursa over cartila3e ca1s

    • (T or M

     – used to better characterize lesions

    Osteochondroma

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    Osteochondroma 

     – :ono1erative

    • Observation alone – indications

    » as0m1tomatic or minimall0 s0m1tomatic cases

     – O1erative

    • Mar3inal resection at base of stal7< includin3 cartila3e ca1 – indications

    » s0m1tomatic lesions• lesion ma0 cause inflammation to surroundin3 tissue

    • lesion ma0 be cosmeticall0 dis1leasin3

     – tr0 to dela0 sur3er0 until s7eletal maturit0

    Multi1le hereditar0 e2ostosis +M!8-

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    Multi1le hereditar0 e2ostosis +M!8-

     – :ono1erative

    • observation – indications

    » most 1atients do not reuire intervention 1rior to reachin3 s7eletal maturit0

     – O1erative

    • sur3ical e2cision of the osteochondroma  – indications

    » dislocated radial heads

    » loss of forearm rotation

     – outcomes

    » sim1le e2cision of the osteochondroma o1timizes chance of im1roved motion

    • Secondar0 chondrosarcoma  – o1erative

    •  wide sur3ical resection – treat same as t01ical chondrosarcoma 

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    (om1lications

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    (om1lications

    • Tendon com1ression – lesions around the shoulder can 3ive rise to

    • rotator cuff im1in3ement

    • subsca1ularis tear

    • bici1ital tendinitis

    • (hondrosarcoma  – in adults< cartila3e ca1 #cm is associated with increased chance of

    mali3nanc0

     – mean a3e of dia3nosis< %0rs

    • seldom in %st decade or after Gth decade of life

    • Bursa formation

    • ecurrence

     – #"GH of cases after resection

    Osteochondroma +a- A 0oun3 3irl 1resented with this lum1 on her le3. t felt bon0 hard. +b- 9"ra0 e2amination

    showed the t01ical features of a lar3e cartila3e ca11ed e2ostosis= of course the cartila3e ca1 does not show on 2 ra0 unless

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    showed the t01ical features of a lar3e cartila3e"ca11ed e2ostosis= of course the cartila3e ca1 does not show on 2"ra0 unless

    it is calcified. The bon0 1art ma0 be sessile< 1edunculated or cauliflower"li7e.

    Osteochondroma ) treatment +a- This #$"0ear"old man had 7nown about the lum1 on his left sca1ula for man0

    0ears. !e sto11ed 3rowin3 at the a3e of %I but the tumour continued to enlar3e. +b- Des1ite the beni3n histolo30 in the

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    bio1s0< the tumour to3ether with most of the sca1ula was removed= sections ta7en from the de1ths of the lesion showed

    at01ical cells su33estive of mali3nant chan3e.

    nicameral Bone (0st

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    nicameral Bone (0st

    • Also referred to as a sim1le bone c0st

    • A non"neo1lastic< serous fluid"filled bone lesion thou3ht to result

    from tem1orar0 failure of medullar0 bone formation near the 1h0sis

    • usuall0 found in 1atients #$ 0ears of a3e

     – location

    • usuall0 found in the 1ro2imal humerus of 0oun3 1atients

    • can be found in other locations includin3 1ro2imal femur< distal tibia< ilium<

    calcaneus< and occasionall0 metacar1als< 1halan3es< or distal radius• arises in the meta1h0sis ad*acent to 1h0sis and 1ro3resses toward the dia1h0sis

     with bone 3rowth

    nicameral Bone (0st

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    nicameral Bone (0st

    • 4ro3nosis – As a 1atient a11roaches s7eletal maturit0< a B( will

    often decrease in size and ma0 heal after 3rowth is

    com1lete

     – Fracture healin3 usuall0 does not lead to c0st resolution

     – euires close follow u1 while in active 1hase due torecurrence and ris7 of fracture or 3rowth arrest

    nicameral Bone (0st

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    nicameral Bone (0st

    • (lassification is im1ortant as it im1actstreatment

    • Active – if c0st is ad*acent to the 1h0sis

    • atent

     – if normal bone se1arates c0st from 1h0sis

    atent B(

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    atent B(

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    NFallen leafJ si3n +1atholo3ic fracture with fallen cortical fra3ment in base of em1t0 c0st is

    1atho3nomonic -

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    1atho3nomonic -

    nicameral Bone (0st

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    nicameral Bone (0st

    • :ono1erative

     – mmobilization alone

    • indications – 1ro2imal humerus lesions with 1atholo3ic fracture +%GH of lesions fill in with native bone

    after acute fracture-

     – As1iration>meth0l1rednisolone acetate in*ection

    • indications – active c0sts +communicates with 1h0sis- in the 1ro2imal humerus

    • techniue – usuall0 reuires several in*ections< es1eciall0 in ver0 0oun3 children

     – bone marrow in*ections have recentl0 been re1orted to be effective

    Sim1le bone c0sts

    +a- A t01ical solitar0 +or unicameral- c0st ) on the shaft side of the 1h0sis and e21andin3

    th t

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    the corte2

    +b- n*ection with meth0l1rednisolone< and +c- healin3. +d- Fracture throu3h a c0st.

    nicameral Bone (0st

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    nicameral Bone (0st

    • O1erative – (uretta3e and bone 3raftin3 >" internal fi2ation based on tumor

    location

    ndications – s0m1tomatic latent c0sts that have not res1onded to steroid in*ections

     – latent c0sts in the 1ro2imal femur that are a structural concern and at ris7 for fracture

    and osteonecrosis

     – 1ro2imal femoral lesions with a 1atholo3ic fracture have a hi3h rate of refracture and

    malunion when treated nono1erativel0 therefore< internal fi2ation is recommended

    • (ontraindications – avoid in active lesions as communication with 1h0sis ma0 lead to 3rowth arrest

    Aneur0smal Bone (0st

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    Aneur0smal Bone (0st

    • A beni3n and non"neo1lastic reactive bone lesion filled withmulti1le blood"filled cavities

     – (an be locall0 destructive to normal bone and ma0 e2tend to soft

    tissue

    • LGH of 1atients are #$ 0rs.

     – ocation

    • #GH in s1ine

    • #$H in lon3 bones +distal femur< 1ro2imal tibia-

    • suall0 in meta1h0sis

    • 4osterior elements of 1elvis

    • Ma0 be found in similar location as telan3iectatic osteosarcomas

    Aneur0smal Bone (0st

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    Aneur0smal Bone (0st

    • adio3ra1hs – e21ansile< eccentric and l0tic lesion with bon0

    se1tae +Jbubbl0 a11earanceJ-

     – usuall0 in meta1h0seal

    • M or (T scan

     – will show multi1le fluid lines

    Aneur0smal Bone (0st

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    Aneur0smal Bone (0st

    Aneur0smal Bone (0st

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    Aneur0smal Bone (0st

    • :ono1erative – :ono1erative fracture mana3ement

    • ndications – AB( with acute fracture

    » indicated until fracture has healed. Once healed< treat as an AB( without fracture unless the fracture has

    led to s1ontaneous healin3 of the AB(

    • O1erative

     – A33ressive curetta3e and bone 3raftin3• ndications

     – s0m1tomatic AB( without acute fracture

    • Techniue – some use ad*uvant treatment +1henol-

    • Outcomes – local recurrence in u1 to #GH and more common in children with o1en 1h0ses

    Ciant (ell Tumor

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    Ciant (ell Tumor

    • A beni3n a33ressive tumor t01icall0 found in the e1i1h0sis of lon3bones

     – more common in females +unli7e most bone tumors which show male

    1redominance-

     – a3es $"G$ 0ears

    • ocation

     – G$H occur around 7nee +distal femur or 1ro2imal tibia-

     – %$H in sacrum and vertebrae +sacrum is most common site in a2ial s7eleton- 

    •  while C(T can rarel0 occur in the s1ine< it usuall0 occurs in the vertebral bod0

     – Distal radius is third most common location

     – 4halan3es of the hand is also a ver0 common location

     – Ma0 arise in the a1o1h0sis +li7e chondroblastoma-

    Ciant"cell tumours The tumour alwa0s abuts

    i h * i i

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    a3ainst the *oint mar3in

    Ciant (ell Tumor

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    Ciant (ell Tumor

    • Mali3nanc0 – 4rimar0 mali3nant 3iant cell tumor

    • metastatic to lun3 in #"GH

    hand lesions have 3reater chance of metastasis

     – Secondar0 mali3nant 3iant cell tumor

    • occurs followin3 radiation or multi1le resections of 3iant

    cell tumor

    8ccentric l0tic e1i1h0seal>meta1h0seal lesion that often e2tends

    into the distal e1i1h0sis and borders subchondral bone

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    into the distal e1i1h0sis and borders subchondral bone

    Ciant (ell Tumor

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    Ciant (ell Tumor

    • :ono1erative – radiation alone

    • indications – onl0 indicated for ino1erable or multi1l0 recurrent lesions

    • outcomes –

    leads to %GH mali3nant transformation – Medical mana3ement

    • indications – medical thera10 can be used to au3ment or re1lace sur3ical mana3ement de1endin3 on the

    s1ecific clinical scenario

     – Bis1hos1honates

    »

    osteclast inhibitors which ma0 decrease the size of the defect in 3iant cell tumors – Denosumab

    » monoclonal antibod0 a3ainst A:K"li3and

    » recent clinical trials su33est denosumab can decrease the size of the bone defect in 3iant

    cell tumor

    Ciant (ell Tumor

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    Ciant (ell Tumor

    • O1erative – 82tensive curetta3e and reconstruction +with ad*uvant

    treatment-

    • ndications – lesions amenable to curretta3e

     – hand lesion treatment is controversial

    » if no cortical brea7throu3h treat with curetta3e and cementin3

    » if si3nificant cortical brea7throu3h consider intercalar0

    resection +with free fibular 3raft- vs. am1utation

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    4MA/ MAC:A:T BO:8

    TMOS

    (hondrosarcoma

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    (hondrosarcoma 

    • Mali3nant chondro3enic lesions can occur in two forms

     – 4rimar0 chondrosarcoma 

    •  which includes – ow"3rade< hi3h"3rade< de"differenitated chondrosarcoma 

     – (lear cell chondrosarcoma – Mesench0mal chondrosarcoma

     – Secondar0 chondrosarcoma 

    • arises from beni3n cartila3e lesions includin3 – Osteochondroma +%H ris7 of mali3nant transfomation-

     – Multi1le hereditar0 e2ostosis +%"%$H ris7 of mali3nant transformation-

     – 8nchondromas +%H ris7 of mali3nant transformation-

     – Olliers disease +#G"$H ris7 of mali3nant transformation-

     – Maffuccis +%$$H ris7 of mali3nant transformation-

    (hondrosarcoma At the a3e of #$ 0ears< this 0oun3 man com1lained of 1ain in the ri3ht

    3roin= 2"ra0 showed an osteochondroma of the ri3ht inferior 1ubic ramus.

    +a- A bio1s0 showed ?beni3n@ cartila3e but a 0ear later the tumour had

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    +a- A bio1s0 showed beni3n cartila3e but a 0ear later the tumour had

    doubled its size +b-< a clear si3n that it was mali3nant

    (hondrosarcoma 

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    ( o d osa co a

    • A3e & location – Found in older 1atients +$"LG 0rs-

     – There is a sli3ht male 1redominance

     – Most common locations include the 1elvis< 1ro2imalfemur< sca1ula

     –

    Tumor location is im1ortant for dia3nosis as thesame histolo30 ma0 be dia3nosed as beni3n in the

    hand but mali3nant if located in the lon3 bones

    (hondrosarcoma 

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    • Crade – IGH of chondrosarcomas are 3rade % or #

     – %GH of chondrosarcomas are 3rade or dedifferentiated chondrosarcoma

    • de"differentiated chondrosarcomas are hi3h 3rade lesions which develo1 from low

    3rade chondroid lesions

    • 4ro3nosis

     – a2ial and 1ro2imal e2tremit0 lesions have a more a33ressive course

     – histolo3ic 3rade correlates with survival

    Crade 5 ,$H survival• Crade 5 R$"L$H survival

    • Crade 5 $"G$H survival

    • De"differentiated chondrosarcoma5 %$H survival

    (hondrosarcoma 

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    • (hondrosarcoma sub"t01es

    • (lear cell chondrosarcoma

     – mali3nant immature cartila3inous tumor accountin3for #H of all chondrosarcomas

     – most common in rd and th decades of life

     –

    1resents as an e1i1h0seal lesion and can bemista7en for low"3rade chondroblastoma 

     – locall0 destructive with 1otential to metastasize

    (hondrosarcoma 

    http://upload.orthobullets.com/topic/8023/images/clear%20cell%202.jpg

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    • Mesench0mal chondrosarcoma 

     – Ma0 occur at several discontinuous sites at

    1resentation and can occur in the soft tissues

     – Treatment includes neo"ad*uvant chemothera10

    followed b0 wide sur3ical resection

    (hondrosarcoma 

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    • adio3ra1hs• 0tic or blastic lesion with reactive thic7enin3 of

    the corte2

     – ow"3rade chondrosarcomas show• Similar a11earance to enchondromas with additional

    cortical thic7enin3>e21ansion and endosteal erosion

     – !i3h"3rade chondrosarcomas show• cortical destruction and a soft tissue mass

    ow"3rade chondrosarcomas

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    3

    (hondrosarcoma 

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    ntra"lesional J1o1cornN mineralization ma0

    be seen

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    be seen

    de"differentiated chondrosarcomas radio3ra1hicall0 show a lower3rade chondroid lesion with su1erim1osed hi3hl0 destructive

    area consistent with the hi3h 3rade transformed dedifferentiated

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    area consistent with the hi3h 3rade transformed dedifferentiated

    chondrosarcoma 

    (hondrosarcoma 

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    • O1erative – intra"lesional curetta3e

    • indications – Crade % lesions

     – treatment of 3rade % lesions located in the 1elvis or a2ial

    s7eleton is controversial

    » man0 authors recommend wide e2cision of all

    chondrosarcomas +even 3rade %- if located in the 1elvis

    (hondrosarcoma 

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     – 'ide sur3ical e2cision• indications

     – 3rade # or lesions

     – some sa0 3rade % lesions in 1elvis

    • historicall0< there is no si3nificant role for radiation or chemothera10

    in t01ical intramedullar0 chondrosarcoma

     – 'ide sur3ical e2cision combined with multi"a3ent

    chemothera10

    • indications – mesench0mal chondrosarcoma 

     – the role of chemothera10 in de"differentiated chondrosarcoma is ver0

    controversial

    ntramedullar0 Osteosarcoma 

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    0

    • ntramedullar0 osteosarcoma is the most common 1rimar0 sarcomaof bone

     – The most common mali3nanc0 of bone is metastatic disease

     – The most common 1rimar0 mali3nanc0 of bone is m0eloma 

     – suall0 occurs in children and 0oun3 adults

    • bimodal distribution of occurrence – ma*orit0 occur in the second decade of life

     – second 1ea7 in occurrence is in elderl0 1atients with 4a3ets disease

     – Most common site is the distal femur and 1ro2imal tibia

     – Other common sites include 1ro2imal humerus< 1ro2imal femur< and 1elvis

    ntramedullar0 Osteosarcoma 

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    0

    • Mali3nanc0 – Most commonl0 dia3nosed as Sta3e B +hi3h 3rade< e2tra"

    com1artmental< no metastases-

     –

    %$"#$H of 1atients 1resent with 1ulmonar0 metastases +obtain (T ofchest-

    • un3 is most common site of metastasis

    • Bone is second most common site

    • Cenetics – 4atients who carr0 the etinoblastoma tumor su11ressor 3ene +b-

    are 1redis1osed to osteosarcoma

    ntramedullar0 Osteosarcoma 

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    0

    • 4ro3nosis – LRH lon3"term survival with modern treatment

     – 4oor 1ro3nostic factors include

    • Advanced sta3e of disease +most 1redictive of survival-

    • es1onse to chemothera10 +as *ud3ed b0 1ercent tumor necrosis of resected

    s1ecimen-

    • Tumor site and size

    • 821ression of 4"3l0co1rotein

    • !i3h serum al7aline 1hos1hatase

    • !i3h lactic deh0dro3enase• 6ascular involvement

    • Sur3ical mar3ins

    • T01e of chemothera10 re3imen

    )haracteristic "lastic and destructie lesion

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    Sun"burst or hair on end 1attern of matri2 mineralization

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    +(odmans Trian3le-

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    Osteosarcoma )1atholo30 +a- After resection this lesion was cut in half= 1ale tumour

    tissue is seen occu10in3 the distal third of the femur and e2tendin3 throu3h the corte2.

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    ntramedullar0 Osteosarcoma 

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    • A %"0ear"old 3irl 1resents with 7nee 1ain for # months es1eciall0at ni3ht. She denies fevers and wei3ht loss. !er 1h0sical e2am

    reveals a 1ainful thi3h mass. A radio3ra1h is shown in Fi3ure A.

    'hat is the ne2t most a11ro1riate ste1 in mana3ment;

    %. e1eat radio3ra1hs in months with observation

    #. 82ternal beam radiation and chemothera10

    . Sur3ical bio1s0 and culture directed intravenous antibiotics

    . :eoad*uvant chemothera10 followed b0 wide e2cision andad*uvant chemothera10

    G. M of the entire bone< whole bod0 bone scan and (T chest

    ntramedullar0 Osteosarcoma 

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    ntramedullar0 Osteosarcoma 

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    O1erative• Multi"a3ent chemothera10 and limb salva3e resection

     – ndications• hi3h 3rade osteosarcoma 

     – (hemothera10• 1reo1erative chemothera10 3iven for I"%# wee7s followed b0 maintenance chemothera10 for R"

    %# months after sur3ical resection

    • ,IH necrosis after neo"ad*uvant chemothera10 is 3ood 1ro3nostic si3n

    • e21ression of multi"dru3 resistance +MD- 3ene 1ortends 1oor 1ro3nosis – tumor cells can 1um1 chemothera10 out of cell with MD e21ression

     – 1resent in #GH of 1rimar0 lesions and G$H of metastatic lesions

    ntramedullar0 Osteosarcoma 

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     – Sur3ical techniue• Trend towards limb salva3e whenever 1ossible

    • Overall survival in osteosarcoma is eual after limb

    salva3e vs. am1utation to deal with local e2tent of

    disease

    • 'ide sur3ical resection

     –

    indications• indicated in low 3rade osteosarcoma such as 1arosteal

    osteosarcoma 

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    Osteosarcoma ) o1erative treatment 4osto1erative 2"ra0s showin3 an

    endo1rosthetic re1lacement followin3 wide resection of the lesion +Stanmore

    m1lants 'orldwide-.

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    1 -

    4arosteal Osteosarcoma 

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    A low 3rade osteosarcoma • More common in females< a3e $"$

     – ocation

    • occurs on surface of meta1h0sis of lon3 bones

    • most common sites include 1osterior distal femur< 1ro2imal tibia< and 1ro2imal humerus – I$H cases occur in the femur

    • marrow invasion occurs in #GH of cases

    • 4ro3nosis

     – ,GH lon3 term survival when local control has been achieved

    • dedifferentiation of 1arosteal osteosarcoma is a 1oor 1ro3nostic factor

    • invasion into the medullar0 cavit0 does not affect lon3"term survival

    !eavil0 ossified< lobulated mass arisin3 from corte2

    a11ears stuc7 onto corte2

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    4arosteal osteosarcoma +a

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    4arosteal Osteosarcoma 

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    • O1erative – 'ide local sur3ical e2cision

    • ndications – standard of care in most 1atients

    • Techniue – man0 consider 3eometric osteotom0 of involved bone to decrease

    lon3 term morbidit0 and retain native *oint

    • (hemothera10 – chemothera10 not indicated unless there is a hi3h 3rade com1onent

    • Outcomes – often curative

    8win3s Sarcoma 

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    • A distinctive small round cell sarcoma • t01icall0 found in 1atients from G"#G 0ears of a3e

    • second most common bone tumor in children

    • uncommon in African Americans and (hinese

     – locations

    • QG$H are found in the dia1h0sis of lon3 bones – the most common locations include 1elvis< distal femur< 1ro2imal

    tibia< femoral dia1h0sis< and 1ro2imal humerus

    • Cenetics

     –  t+%%5##- translocation found in all cases

    8win3s Sarcoma 

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    • 4ro3nosis – Survival

    • R$"L$H lon3 term survival with isolated e2tremit0 disease at 1resentation and

    a11ro1riate treatment>tumor res1onse to chemothera10

    • $H lon3 term survival with 1elvis lesions

    • %GH lon3 term survival if 1atient 1resents with metastatic disease

     – 4oor 1ro3nostic factors

    • s1ine and 1elvic tumors

    • tumors 3reater than %$$cm

    • ,$H necrosis with chemothera10

    • elevated lactic deh0dro3enase levels

    • 1G mutation in addition to t+%%5##- translocation

    8win3s Sarcoma 

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    • 4resentation – 1ain often accom1anied b0 fever

     – often mimics an infection

    • 4h0sical e2am

     – swellin3 and local tenderness

    ar3e destructive lesion in the dia1h0sis or

    meta1h0sis with a moth"eaten a11earance

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    1 0 11

    4eriosteal reaction ma0 3ive Jonion

    s7inJ or JsunburstJ a11earance

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    11

    8win3@s tumour 82am1les of 8win3@s tumour in

    +a- the humerus< +b- the mid"shaft of the fibula and +c- the lower end of the fibula.

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    8win3s Sarcoma 

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    • abs – 8S is elevated

     – 'B( is elevated

     –

    anemia is common – lactic deh0dro3enase

    Bone marrow bio1s0 – reuired as 1art of wor7u1 for 8win3s to rule out

    metastasis to the marrow

    8win3s Sarcoma 

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    • O1erative

     – chemothera10 and limb salva3e resection

    • indications – standard of care in most 1atients

    • chemothera10 – 1reo1erative chemothera10 3iven for I"%# wee7s followed b0 sur3ical resection and

    maintenance chemothera10 for R"%# months

    • irradiation – current trend is towards sur3ical resection and awa0 from irradiation due to lon3

    term morbidit0 associated with radiation – situations where radiation ma0 be used

    » non"resectable tumors +e3. lar3e s1inal tumors-

    » 1atients who 1resent with widel0 metastatic disease

    8win3s Sarcoma 

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    • Small"round"cell tumor differential +b0 a3e- – G 0rs5 neuroblastoma or leu7emia 

     – G"%$ 0rs5 eosino1hilic 3ranuloma 

     –

    G"$ 0rs5 8win3s sarcoma  – $ 0rs5 l0m1homa 

     – G$ 0rs5 m0eloma 

    Multi1le M0eloma 

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    A neo1lastic 1roliferation of 1lasma cells that 1resents withs7eletal lesions

     – neo1lastic 1lasma cells 1roduces immuno3lobulins

    • heav0 chains5 3C +G#H-< 3A +#%H-< 3M +%#H-

    • li3ht chains5 7a11a or lambda

     – a7a Bence Pones 1roteins

    • Disease forms

     – disease ta7es multi1le forms that var0 in treatment and 1ro3nosis and

    includes

    • multi1le m0eloma

    • solitar0 1lasmac0toma 

    • osteosclerotic m0eloma 

    Multi1le M0eloma 

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    • Bone scans are cold in $H so obtain a s7eletal

    surve0

    M0eloma The characteristic 2"ra0 features are bone rarefaction< vertebral

    com1ression fractures< e21andin3 lesions +t01icall0 in the ribs and 1elvis- and

    1unched"out areas in the s7ull and the lon3 bones.

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    Multi1le M0eloma 

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    Serum labs – anemia 

     – elevated creatinine

     – h01ercalcemia 

    • 1resent in $H of 1atients due to e2cessive resor1tion of bone

     – 8S often elevated – S484 +serum 1rotein electro1horesis-

    • M s1i7e 1resent +G$H 3C< #GH 3A-

    • rine

     – 1roteinuria 

     – 484 +urine 1rotein electro1horesis-

    • ma0 show Bence Pones 1roteins +secreted immuno3lobulin 7a11a and lambda li3ht

    chains-

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    M8TASTAT( BO:8 DS8AS8

    Metastatic (ancer of Bone

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    • Metastatic cancer is the most common reasonfor a destructive bone lesion in adults

     – carcinomas that commonl0 s1read to bone include

    breast• lun3

    • th0roid

    • enal

    • Adrenal• 4rostate

    Metastatic (ancer of Bone

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    Bone is the third most common site for metastaticdisease +behind lun3 and liver-

    • (ommon sites of metastatic lesions include

     – A2ial s7eleton +vertebral bodies< 1elvis< ribs-» thoracic s1ine is most common site of bon0 metastasis

     – 4ro2imal limb 3irdle

    » 1ro2imal femur is most common site of fracture secondar0

    to metastatic bone lesions

    Metastatic (ancer of Bone

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    • 4ro3nosis• Median survival in 1atients with metastatic bone

    disease

     – Th0roid5 I months

     – 4rostate5 $ months

     – Breast5 # months

     –

    Kidne05 variable de1endin3 on medical condition but ma0 beas short as R months

     – un35 R months

    Metastatic (ancer of Bone

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    • 6ascular s1read 5• Batsons vertebral 1le2us

     – valveless venous 1le2us of the s1ine that 1rovides a route

    of metastasis from or3ans to a2ial structure

    includin3 vertebral bodies< 1elvis< s7ull< and 1ro2imal limb3irdles

    Arterial tree metastasis – mechanism b0 which lun3 and renal cancer s1read to the

    distal e2tremities

    Metastatic (ancer of Bone

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    • 4url0 l0tic or mi2ed l0tic>blastic lesions

     – lun3< th0roid< and renal are 1rimaril0 l0tic

     – R$H of breast (A is blastic – ,$H of 1rostate (A is blastic

    (ortical metastasis are common in lun3

    cancer

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    esions distal to elbow and 7nee are usuall0

    from lun3 or renal 1rimar0

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    Metastatic (ancer of Bone

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    Coal of treatment in metastatic disease is 1ain control andmaintainence of 1atient inde1endence

    • Bis1hos1honate thera10

    • Stabilization of com1lete fracture< 1osto1erative radiation

    • 4ro1h0lactic stabilization of im1endin3 fracture< 1osto1erative

    radiation

    • 4reo1erative embolization ) enal > Th0roid

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    S0novial (hondromatosis

    A11le (ore A11earance

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    4i3mented 6illonodular S0novitis

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    • 46:S is an idio1athic monoarticular reactive s0novial disease

     – characterized b0 e2uberant 1roliferation of s0novial villi and nodules

    • most commonl0 in adults a3e $"G$ but can occur at an0 a3e

     – location• ma0 be localized +intra"articular or classic form-

    • 7nee is the most common site of involvement +I$H- – other involved sites include hi1< shoulder< and an7le

    • can be diffuse +e2tra"articular e2tension- –  when e2tra"articular 7nown as 3iant cell tumor of tendon sheath

    » occurrin3 alon3 tendon sheaths of hands and feet

    • 4athoanatom0 and etiolo30

     – half of 1atients re1ort 1rior histor0 of trauma to afflicted re3ion• thou3ht to be a reactive 1rocess

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    4i3mented 6illonodular S0novitis

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    Arthrocentesis – 3rossl0 blood0 effusion

    • Arthrosco10 +3ross a11earance-

     – brownish or reddish inflamed s0novium is t01ical of 46:S

    4i3mented 6illonodular S0novitis

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    O1erative –  total s0novectom0

     – intra"articular disease

    » techniues ran3e from arthrosco1ic 1artial s0novectom0 to full0 o1en

    total s0novectom0

    » de1endent on e2tent and location of disease» freuent recurrence is common

    » mostl0 due to incom1lete s0novectom0

     – e2tra"articular

    » mar3inal e2cision is adeuate for 3iant cell tumor of tendon sheath

    »

    recurrence +which is common- is treated with re1eat e2cision