Bone tumors

Classification Primary a. Benign b. Malignant Secondary (metastases) General Principles of Radiological Diagnosis Age Solitary or Multiple Sit of n!ol!ement "ocation #it$in t$e Bone Margins Soft %issue &'tension Benign !s. Malignant Benign( )on Cancerous Malignant( Cancerous

Benign Tumors
1. ENOSTOSIS:
Single/ multiple Uniformly dense Always medullary in location Narrow zone of transition DD- osteoblastic metastases

2. OSTEOMA: Slow growing Arises from skull, paranasal sinuses and mandible Broad base wit well defined margins!

"ardners syndrome

3. OSTEOID OSTEOMA: #ale preponderance $nd to %rd decade Diap ysis of long bones! &ound or o'al area of translucency wit sclerotic margin and nidus!

DD-(steoblastoma ) ronic sclerosing osteomyelitis!

4. OSTEOBLASTOMA:
#ales, second decade Similar to osteoid osteoma but *$cms Spine + flat bones ,ell defined radiolucency in corte- or medulla wit cortical e-pansion !

5.EXOSTOSES: (sseous outgrowt arising from bone corteArises in tubular bones,near t e metap ysis )artilage cap ., t ickness /-0mms! )omplication-) ondrosarcoma

6. ENCHONDROMA: (riginates in t e medullary ca'ity )ommonly in t e p alanges S arply defined lytic lesion wit endosteal scalloping

1ew specks of calcifications #ultiple in (llier2s disease

7.MAFFUCIS SYNDROME

#ultiple enc ondromas . aemangiomas

8. CHONDROBLASTOMA: /3-$3 4ears 5pip ysis ,ell defined, radiolucent,o'al lesion,wit t in rim sclerosis and cortical e-pansion! Stippled calcification may present of

9.

!ANT CELL T"MO"R: $3-63 45A&S 7redilection for bones ad8acent to t e knee and distal end of radius! 5ccentrically located zone of translucency, beneat t e articular corte-

Soap-Bubble Appearance DD-Aneurysmal bone cyst ) ondroblastoma

1#.HEAMAN !OMA: Solitary 9 oracic or :umbar 'ertebrae ;ncreased translucency wit fine 'ertical striations! )9< 7olka dot appearance

11. NON OSS!$%!N $!BROMA: 5ccentric in t e medullary ca'ity /3-$3 yrs Around knee =ray< well marginated eccentric geograp ic lesion in t e metadiap ysis wit t in rim of sclerosis DD-1ibrous dysplasia Solitary bone cyst

1&. SOL!TAR% BONE C%ST: ) ild ood / adolescence

Always unilocular 7ro-imal umerus and femur #etadiap ysealp yseal in location = >ray <welldefined lytic lesion, sclerotic borders, t inning of corteDD-) ondroma

1'. ANE"R%SMAL BONE C%ST:

?$3yrs 7redilection for long bones + D: spine = ray < Area of bone resorption wit t in,e-panded corteDD-"iant cell tumours

14. !NTRAOSSEO"S L!(OMA: Arises in t e medulla ,ell defined lytic lesion wit s arp margins! )entral ossified nodule

MAL! NANT BONE T"MORS

1. CHONDROSARCOMA: *63yrs, $</ 7el'is, ribs, pro- femur and umerus 9ypes< )entral > intramedullary epicenter 7erip eral > from surface of bone 5-pansile osteolytic ragged looking lesion wit calcifications and soft tissue in'ol'ement

&.CHORDOMA: 7redilection for sacral and cranial regions 63 > @3 yrs, $</ Sacrum< :ytic,o'al or lobulated wit relati'ely well defined margins and a large soft tissue mass, amorp ous caln .

Sp eno-occipital region< dorsal aspect of sella and cli'us destroyed wit a soft tissue mass indenting on t e nasop aryngeal air space

'. E)!N S SARCOMA: )entral diap yseal lytic lesion A-%3 years Diap ysis #edullary in location 7ermeati'e destruction :amellated / onion periosteal reaction )odman2s triangle :arge well defined soft tissue mass peel

4. M"LT!(LE M%ELOMA: #ost common primary neoplasm of bone

Disease of t e elderly, *A3yrs ,ell defined punc ed out lytic lesions all o'er skeleton DD- #etastases

4. (LASMOC%TOMA: Solitary myeloma 9 oracolumbar )an also occur in pel'is,femur and umerus ,ell defined,s arply demarcated and c aracteristically wit out sclerotic reaction

*. L%M(HOMA: ,ide range of incidence Bip asic distribution ;n'ol'es diap ysis of long bones and also pel'is :ytic,permeati'e destruction!

6. ADAMANT!NOMA: 5ccentric location Diap ysis ,ell circumscribed, multiloculated Slig tly e-pansile wit cortical t inning!

7. OSTEOSARCOMA Definition: Ba malignant primary neoplasm of bone composed of a malignant connecti'e tissue stroma wit e'idence of malignant osteoid, bone or cartilage formation! B9ype of bone cancer t at de'elops in t e cells Cforms t e outer co'ering of t e boneD! BAE of all c ild ood cancers! B)ommon sites< long bones, knee, upper leg, t ig bone, lower leg and upper arm! B(steosarcoma is a cancerous CmalignantD bone tumor t at usually de'elops during t e period of rapid growt t at occurs in adolescence, as a teenager matures into an adult! A. .A: (steogenic Sarcoma

In!i"en!e: B#ore common in male adolescents B)ommon occurs between ages /3 and $A years old

Ri#$ %n" &'e"i#(o#in) F%!to'#: B9 e cause is not known! B;n some cases, osteosarcoma runs in families, and at least one gene as been linked to increased risk! B9 is gene is also associated wit familial retinoblastoma, a cancer of t e eye t at occurs in c ildren! 7resence of certain rare, in erited cancers, suc as t e following< Li*F'%+,eni #-n"'o,e - a rare family predisposition to multiple types of cancers Csuc as soft tissue sarcomas, breast cancer, brain tumors, osteosarcoma, leukemia, melanoma, cancer of t e adrenal corte-, and ot ersD caused by a mutation in a gene t epA% tumor-suppressor gene - t at normally curbs cancer! 'etino./%#to,% - a malignant tumor of t e retina t at usually occurs in c ildren younger t an 6 years old! Bteenage growt spurts Bbeing tall for a specific age Bpre'ious treatment wit radiation for anot er cancer, especially at a young age or wit B;n8ury B;nfection B#etabolic or ormonal disturbance BFig fat diet Cfor adultsD B:ack of e-ercise Cfor adultsD ig doses of radiation Bpresence of a benign Cnon-cancerousD bone disease

BSmoking Cfor adultsD BDrinking alco ol Cfor adultsD M%nife#t%tion#: B:ocalized pain at t e site of t e tumor BSwelling, warmt , and redness caused by t e 'ascularity of t e tumor B)oug BDyspnea BA c ild may limp or suffer a pat ologic fracture B9rauma may call attention to a pre-e-isting tumor BBone fracture Cmay occur after w at seems like a routinemo'ementD BBone pain B:imitation of motion B7ain w en lifting Cif t e tumor is in t e armD B#uscle atrop y St%)e# of o#teo#%'!o,% B(nce osteosarcoma as been found, more tests may be done to find out if cancer cells a'e spread to ot er parts of t e body! 9 is is called staging! BAt present, t ere is no staging system for osteosarcoma! B;nstead, most patients are grouped depending on w et er cancer is found in only one part of t e body Clocalized diseaseD or w et er t e cancer as spread from one part of t e body to anot er Cmetastatic diseaseD! B9 e following groups are used for osteosarcoma< /! :ocalized osteosarcoma 9 e cancer cells a'e not spread beyond t e bone or nearby tissue in w ic t e cancer began! $! #etastatic osteosarcoma 9 e cancer cells a'e spread from t e bone in w ic t e cancer began to ot er parts of t e body! 9 e cancer most

often spreads to t e lungs! ;t may also spread to ot er bones! ;n multifocal osteosarcoma, tumors appear in $ or more bones, but a'e not spread to t e lungs! %! &ecurrent &ecurrent disease means t at t e cancer as come back CrecurredD after it as been treated! ;t may come back in t e tissues w ere it first started or it may come back in anot er part of t e body! (steosarcoma most often recurs in t e lung! , en osteosarcoma recurs, it is usually wit in $ to % years after treatment is completed! :ater recurrence is possible, but rare! Diffe'ent t-(e# of o#teo#%'!o,% 9 ere are many different types of osteosarcoma t at are Guite different in natural istory and t eir potential for let al growt ! 9 e HgradeI is a term used to indicate t e le'el of aggressi'e growt and potential for metastasis, usually based upon t e appearance of t e tumor under microscopic analysis! /! C/%##i!%/ o' !on0ention%/ 1i)1 )'%"e !ent'%/ t-(e , ic accounts for J3E of all osteosarcomas! By far t e most common osteosarcoma! Se'eral ot er unusual ig grade types $! Te/%n)ie!t%ti! O#teo#%'!o,% 9elangiectatic osteosarcoma is a rare and aggressi'e 'ariant of osteosarcoma! ;t accounts for AE of osteosarcomaK and arises in t e metap ysis and diap ysis! ;t presents more commonly as a pat ological fracture t an con'entional osteosarcoma! 9elangiectatic osteosarcoma is often entirely osteolytic on plain --ray!

%! S,%// !e// O#teo#%'!o,% 9 is rare subtype of osteosarcoma accounts for less t an /E of all osteosarcoma tumors! 9 is tumor occurs in t e metap ysis of t e long bones! 7atients present wit local pain and swelling! (n --rays, a blastic lesion it e-tends into t e s aft of t e bone wit a permeati'e destructi'e pattern is seen! 9 ese tumors a'e a poor prognosis! ) emot erapy and surgery are used! 6! Se!on"%'- Cassociated wit anot er pre-e-isting disease suc as 7aget2s disease or prior radiation e-posureD type! 9 ere are low grade types of osteosarcoma too! A! S+'f%!e o' 2+3t%!o'ti!%/ /o4 )'%"e o#teo#%'!o,% Surface osteosarcomas are osteosarcomas w ose epicenters are outside t e corte- of t e underlying bone! 9 ey usually arise in relation to t e periosteum or t e corte- of t e bone wit minimal or no in'ol'ement of t e medullary ca'ity! S+. t-(e# of S+'f%!e o#teo#%'!o,%: a. &%'o#te%/ O#teo#%'!o,% t e most common form of surface osteosarcoma ;t as a uniGue presentation and anatomic distribution because @AE to L3E of cases arise as densely radiopaGue masses attac ed to t e distal posterior femur y gi'e rise to a sensation of fullness, pre'ent complete range of fle-ion, and e'en be percei'ed as a mass t roug t e o'erlying soft tissues, it is unusual for t e entity to be spontaneously painfu b. &%'io#te%/ O#teo#%'!o,% less common t an parosteal osteosarcoma as a matri- component t at is mainly cartilaginous Alt oug t e lesion is associated wit t e bone surface, it

tends to arise between t e corte- and t e cambium layer of t e periosteum, so t at t ere often is a periosteal reaction 'isible radiograp ically!

9 ere also often is underlying cortical t ickening or erosion, and it usually occurs along t e tibial or femoral diap ysis rat er t an posterior to t e metap ysis of t e distal femur!

c. De"iffe'enti%te" &%'o#te%/ O#teo#%'!o,% - ig -grade osteosarcoma arising in t e clinical setting of recurrent parosteal osteosarcoma of t e usual low-grade 'ariety! d. 5i)1*6'%"e S+'f%!e O#teo#%'!o,% - 9 is tumor, w ic manifests as a surface lesion of bone, is entirely ig grade istologically - ;t may appear radiograp ically as an ordinary low-grade parosteal osteosarcoma wit dense sclerosis! - Alternati'ely, it may a'e mi-ed sclerosis and radiolucency, or, occasionally, it may form a soft tissue mass wit relati'ely little radiodensity - Because it is a ig er grade lesion t an ordinary parosteal osteosarcoma, its local growt and aggressi'eness are more accelerated - )onseGuently, patients wit t is disease are more likely to a'e more distressing symptoms and signs t an t ose wit usually low-grade parosteal osteosarcomas! 0! Lo4 )'%"e !ent'%/ o#teo#%'!o,%. 9 is unusual 'ariant of osteosarcoma constitutes a 'ery small percentage of osteosarcomas! ;ts microtrabecular osseous matriarc itecture in a bland fibrous stroma bears some resemblance to fibrous

dysplasia and ot er benign lesions, but most often it resembles t e istologic features of low-grade parosteal osteosarcoma! ;f bone formation is scant, t ere also is a istologic resemblance to desmoplastic fibroma, and $ reported cases were misdiagnosed initially as 7aget disease!

Ot1e' t-(e# of O#teo#%'!o,%: @! E(it1e/ioi" O#teo#%'!o,% 5pit elioid osteosarcoma is an osteosarcoma in w ic t e tumor cells are so poorly differentiated t at it is difficult to determine istologically w et er t e tumor is a sarcoma or a carcinoma! L! O#teo./%#to,%*/i$e O#teo#%'!o,% (steoblastoma-like osteosarcoma resembles osteoblastoma istologically in t at it produces t e same sort of microtrabecular bone lined by osteoblasts as does osteoblastoma J! C1o,"'o./%#to,%*/i$e O#teo#%'!o,% 9 is type of osteosarcoma is best distinguis ed by its osteoid or bone formation, atypical mitotic acti'ity, and infiltration of ad8acent intertrabecular space /3. 6i%nt !e//*'i!1 O#teo#%'!o,% About $AE of osteosarcomas contain benign multinucleated giant cells resembling osteoclasts! &arely, an osteosarcoma may contain so many benign giant cells t at t e malignant elements in t e background are obscuredM in t ese cases, t e lesion may be mistaken istologically for a giant cell tumor! 9 is is more apt to occur in t e sacrum, w ere giant cell tumor is muc more common t an osteosarcoma and t e radiograp ic differences between giant cell tumor and

osteosarcoma are less apparent t an in long bones, w ere t e radiograp ic differences are more con'entional and osteosarcomas are more common //! 6n%t1i! O#teo#%'!o,% (steosarcomas of t e mandible and ma-illa usually manifest wit swelling or pain! &adiograp ically, t ey often are radiolucent or a mi-ture of radiolucent and radiodense areas! /$! M+/tifo!%/ O#teo#%'!o,% 9 is e-tremely unusual condition affects multiple osseous sites simultaneously at t e time of presentation Csync ronous formD or multiple skeletal sites at 'arying inter'als Cmetac ronous typeD /%! Int'%!o'ti!%/ O#teo#%'!o,% 'ery rare anatomic 'ariant of osteosarcoma, a ig -grade osteosarcoma entirely confined wit in t e bony corte-! ;t usually manifests as an area of cortical radiolucency wit perilesional sclerosis! Depending on its size, it may be confused wit osteoid osteoma and osteoblastoma, but its cellular atypia and local aggressi'eness distinguis it from t ose entities /6! E3t'%#$e/et%/ O#teo#%'!o,% (steosarcoma in an e-traskeletal site accounts for fewer t an $E of all soft tissue sarcomas! Unlike con'entional osteosarcoma, it tends to occur in late adult ood, and most patients are in t e fift to se'ent decades at t e time of diagnosis! #ost cases arise in t e deep soft tissues wit a predilection for t e t ig followed by t e buttocks, upper e-tremities, and t e retroperitoneum! 7atients usually a'e an enlarging soft tissue mass, and t e mass may be painful &%t1o(1-#io/o)-:

A!(steosarcoma occurs mainly in t e metap yses of long bones, sites of acti'e epip yseal growt ! Distal femur 7ro-imal tibia 7ro-imal umerus B! As a tumor of mesenc ymal cells, osteosarcoma demonstrates productionof osteoid cells ) !;t is a bulky tumort at e-tends beyond t e bone t e bone into a soft tissue! D! 9 is may encircle t e bone and destroy t e trabeculae of affected area! 5! (steosarcoma disseminates t roug bloodstream, usually to t e lung! 1! (t er sites of metastatic spread include ot er bones and 'isceral organ S!1e,%ti! Di%)'%,

Predisposing *actors
+ig$ grade mesenc$ymal tumor Distal *emur Pro'imal %ibia Pro'imal +umerus *ormation of ,steoid Bul-y tumor t$at destroys trabeculae of diseased area "ungs/ Bones/ Metastasi.e t$roug$ 0isceral organs blood streams

Di%)no#ti! St+"ie# BBone #arrow aspiration B;ncreased serum alkaline p osp atase BBlood tests BBone scan to see if t e cancer as spread to ot er bones B)9 scanof t e c est to see if t e cancer as spread to t e lungs B)9 scan of t e affected area B(penbiopsyCat time of surgery for diagnosisD B=-ray of t e affected area M%n%)e,ent: #edical< B) emot erapy Fig -dose met otre-ate wit leuco'orin citro'orum factor rescue Do-orubicin CAdriamycinD combinations of bleomycin, actinomycin, cyclop osp amideC)yto-anD, ifosfamideC;fe-D, and cisplatin are used )arboplatin C7araplatinD Surgical< BAmputation of affected e-tremity

B;n most cases, surgery can remo'e t e tumor w ile sparing t e affected limb Ct is is called limb-sal'age surgeryD! Lo!%/i7e" O#teo#%'!o,% 9reatment of localized osteosarcoma may in'ol'e c emot erapy, followed by surgery, w ic is t en followed by ad8u'ant c emot erapy!

Met%#t%ti! O#teo#%'!o,% #etastatic disease is cancer t at as spread from t e place in w ic it started to ot er parts of t e body! 9reatment for metastatic osteosarcoma may include<

emot erapy, followed by surgery to remo'e t e cancer, w ic is t en followed by ad8u'ant c emot erapy

Surgery Surgery

to remo'e t e cancer, followed by ad8u'ant c emot erapy to remo'e cancer t at as spread to t e lungs!

Re!+''ent O#teo#%'!o,% 9reatment for recurrent osteosarcoma will depend on<

, , (t

ere t e cancer recurred at kind of treatment was gi'en pre'iously er factors!

;n some cases, a clinical trial may be a reasonable treatment option! ;f t e cancer as only come back in t e lungs, treatment may entail surgery to remo'e t e

cancer in t e lungs wit or wit out c emot erapy! ;f t e cancer as come back in ot er places besides t e lungs, treatment may entail combination c emot erapy!

A##e##,ent /! $! %! 6! A! 0! 5ncourage patient to discuss problem and course of symptoms! Note patient and family2s understanding of t e disease, coping wit t e problem and management of pain! 7alpate mass gently on p ysical e-amination! Note size and associated soft-tissue swelling, pain and tenderness of t e mass! Assess neuromascular status and range of motion e-tremity! 5'aluate motility and ability to perform acti'ities of daily li'ing!

N+'#in) Di%)no#i#: Acute pain related to p ysical in8uring agents ;neffecti'e breat ing pattern related to musculoskeletal impairment ase'idenced by usage of accessory muscles in breat ing Acti'ity intolerance related to pain ;mpaired social interaction related to limited p ysical mobility ;neffecti'e role performance related to body image alterationM p ysicalillness N+'#in) Inte'0ention /! $! %! 6! A! 0! 7ro'ide Guiet en'ironment and calm acti'ities to pre'ent or lessen pain! 7ro'ide comfort measure suc as back rub, c ange position and use of eat or cold application! 5ncourage di'ersional acti'ities Administer analgesics as indicated to ma-imal dose as needed! 5ncourage t e patient to increase fluid intake! 5ncourage rest periods to pre'ent fatigue!

@! L! J! /3!

7ro'ide accurate information about t e situation, medication and treatment! Assess muscle strengt , gross and fine motor coordination! 7ro'ide pillows for cus ion and support! Neep side rails up all t e time!

Co,(/i!%tion#

Alopecia &eduction in number of leucocytes and platelets Septicemia Bleeding Anemia Nidney damage Fearing loss

I//+#t'%tion#:

Con0ention%/ O#teo#%'!o,%

Te/%n)ie!t%ti! O#teo#%'!o,%

S,%// !e// o#teo#%'!o,%

E(it1e/ioi" o#teo#%'!o,%

O#teo./%#to,%*/i$e o#teo#%'!o,%

C1on"'o./%#to,%*/i$e o#teo#%'!o,%

6i%nt !e//*'i!1 o#teo#%'!o,%

6n%t1i! o#teo#%'!o,%

Lo4 )'%"e !ent'%/ o#teo#%'!o,%

&%'o#te%/ o#teo#%'!o,%

&%'io#te%/ o#teo#%'!o,%

De"iffe'enti%te" (%'o#te%/ o#teo#%'!o,%

5i)1 )'%"e #+'f%!e o#teo#%'o,%

M+/tifo!%/ o#teo#%'!o,%

Int'%!o'ti!%/ o#teo#%'!o,%

E3t'%#$e/et%/ o#teo#%'!o,%