BISMILALLAH ALREHMAN BISMILALLAH ALREHMAN ALAHEEMALAHEEM

DR SARDAR SAJID MEHMOOD KHANDR SARDAR SAJID MEHMOOD KHANMBBS, MRCS (Ireland), FCPS (Ortho)MBBS, MRCS (Ireland), FCPS (Ortho)

SENIOR REGISTRAR, RIYADH CARE HOSPITALSENIOR REGISTRAR, RIYADH CARE HOSPITAL

OSTEOSARCOMAOSTEOSARCOMA

Osteosarcoma is primary malignant bone Osteosarcoma is primary malignant bone forming tumorforming tumor

Common in young children and adolescents at Common in young children and adolescents at growing ends of bonesgrowing ends of bones

OSTEOSARCOMAOSTEOSARCOMA

HIST0RYHIST0RY

An ancient disease present in Greek, Egyptian An ancient disease present in Greek, Egyptian & Indian literature.& Indian literature.

SARCOMA derived from Greek roots, means SARCOMA derived from Greek roots, means fleshy excrescence, used first time by English fleshy excrescence, used first time by English Surgeon, JOHN ABERNATHY in 1804.Surgeon, JOHN ABERNATHY in 1804.

OSTEOSARCOMA, first time used by French OSTEOSARCOMA, first time used by French Surgeon, Alexis Boyer in 1805Surgeon, Alexis Boyer in 1805

(PERSONEL SURGEON TO NAPOLEON) (PERSONEL SURGEON TO NAPOLEON)

HISTORYHISTORY 1847,BORON GUILLAUME DUPUYTREN 1847,BORON GUILLAUME DUPUYTREN

demonstrated gross pathologic appearance asdemonstrated gross pathologic appearance as

Osteosarcoma is a true cancerous degeneration Osteosarcoma is a true cancerous degeneration of bone, manifest itself in the form of white or of bone, manifest itself in the form of white or reddish mass, lardaceous and firm at an early reddish mass, lardaceous and firm at an early stage of disease but presenting at latter stage stage of disease but presenting at latter stage points of softening, cerebriform matter, points of softening, cerebriform matter, extravasating blood, and white or straw extravasating blood, and white or straw colored fluid of viscid consistence in its colored fluid of viscid consistence in its interior.interior.

HISTORYHISTORY

1900,VITTORIO PUTTI,SCAGLIETTI & 1900,VITTORIO PUTTI,SCAGLIETTI & COMPANACCI from ITLAY shaped treatment for COMPANACCI from ITLAY shaped treatment for Bone Tumor.Bone Tumor.

1921,ERNEST AMROY CODMAN &JAMES 1921,ERNEST AMROY CODMAN &JAMES EWING created registry for Bone Sarcoma.EWING created registry for Bone Sarcoma.

1950,HENRY L.JAFFE,LOUIS LICTENSTEIN 1950,HENRY L.JAFFE,LOUIS LICTENSTEIN established all key histological criteria used to established all key histological criteria used to diagnose most of Bone Tumors.diagnose most of Bone Tumors.

HIST0RYHIST0RY

1970,NORMAN JAFFE, introduced 1970,NORMAN JAFFE, introduced Chemotherapeutic agents such as Chemotherapeutic agents such as METHOTREXATE & ADRIAMYCIN, METHOTREXATE & ADRIAMYCIN, dramatically improved the treatment of dramatically improved the treatment of Osteosarcoma through their ability to treat Osteosarcoma through their ability to treat Micro metastasis.Micro metastasis.

1976,ROSEN introduced Custom made 1976,ROSEN introduced Custom made Prosthesis.Prosthesis.

HISTORYHISTORY

1980,ENNEKING1980,ENNEKING

Surgical Staging of Musculoskeletal Surgical Staging of Musculoskeletal Sarcomas.Sarcomas.

INCIDENCEINCIDENCE

Sixth leading cancer under age of 15Sixth leading cancer under age of 15 In USA 400 cases per Year. In USA 400 cases per Year.

(4.8 per Million <20 Y ), (4.8 per Million <20 Y ), More common in black Americans More common in black Americans

Male 5.2 per Million per year, Male 5.2 per Million per year, Female 4.2 per Million per year Female 4.2 per Million per year

Rare below 5 year(0.5 per Million per year)Rare below 5 year(0.5 per Million per year) 300 die in each year300 die in each year 22NDND peak in incidence in Elderly, associated with Paget peak in incidence in Elderly, associated with Paget

disease, Bone infarct, or Post radiation.disease, Bone infarct, or Post radiation.

Common sites

LOCATIONLOCATION

FEMUR FEMUR 42%(75% DISTAL)42%(75% DISTAL)

TIBIA TIBIA 19%(80% PROXIMAL)19%(80% PROXIMAL)

HUMERUS HUMERUS 10%(90% PROXIMAL)10%(90% PROXIMAL)

SKULL &SKULL & JAW JAW 8%8%

PELVIS PELVIS 8%8%

DISTAL FEMURDISTAL FEMUR

Most common site

PROXIMAL TIBIAPROXIMAL TIBIA

PROXIMAL HUMERUSPROXIMAL HUMERUS

DISTAL RADIUSDISTAL RADIUS

ILEUMILEUM

Metastasize to Metastasize to sacrum,sacrum, contra lateralcontra lateral ileum,ileum, hip hip

LUMBER VERTEBRALUMBER VERTEBRA

MANDIBLEMANDIBLE

RIBRIB

SCAPULASCAPULA

SKULLSKULL

ETIOLOGYETIOLOGY

Exact cause is unknownExact cause is unknown Risk factorsRisk factors

1;Rapid Bone Growth1;Rapid Bone Growth

2; 2; RadiationRadiation

3;Genetic predisposition3;Genetic predisposition

ETIOLOGYETIOLOGY

Bone Dysplasias, including Paget Disease, Fibrous Dysplasias, Enchondromatosis, Hereditary Multiple Exostosis & Retinoblastoma (Germ Line)

*Mutations in Tumor Suppressor Genes,P53 & Rb have major role in Osteosarcoma development

PATHOLOGYPATHOLOGY

PATHOLOGYPATHOLOGY

Localized at Metaphyseal ends of Long BonesLocalized at Metaphyseal ends of Long Bones Solid, Hard, Irregular due to Tumor Spicules of Solid, Hard, Irregular due to Tumor Spicules of

Calcified Bone, Surrounding Tissue infiltratedCalcified Bone, Surrounding Tissue infiltrated

PATHOLOGYPATHOLOGY

Microscopically, Tumor Cells are Pleomorphic,some Microscopically, Tumor Cells are Pleomorphic,some are Giant with numerous Mitosis, producing are Giant with numerous Mitosis, producing Amorphous, Eosinophilic Osteoid with or without Amorphous, Eosinophilic Osteoid with or without central Calcification.central Calcification.

HISTOLOGICAL VARIANTHISTOLOGICAL VARIANT

OsteoblasticOsteoblastic ChondroblasticChondroblastic FibroblasticFibroblastic Ana plasticAna plastic TelangiectaticTelangiectatic

CLINICAL VARIANTS CLINICAL VARIANTS PeriostealPeriosteal ParostealParosteal MultifocalMultifocal SynchronousSynchronous MetachronousMetachronous

TelangiectaticTelangiectatic SecondarySecondary RadiationRadiation Paget DiseasePaget Disease Bone DysplasiasBone Dysplasias

ClassicalClassical

MetaphysealMetaphyseal OsteoscleroticOsteosclerotic SunburstSunburst Codman TriangleCodman Triangle

PAROSTEAL OSTEOSARCOMAPAROSTEAL OSTEOSARCOMA

PERIOSTEAL OSTEOSARCOMAPERIOSTEAL OSTEOSARCOMA

DiaphysealDiaphyseal Cortical encirclingCortical encircling High gradeHigh grade

TELANGIECTATIC TELANGIECTATIC OSTEOSARCOMAOSTEOSARCOMA

RadiolucentRadiolucent Little Periosteal reactionLittle Periosteal reaction

RADIOLOGICAL DIFFERENTIAL RADIOLOGICAL DIFFERENTIAL DIAGNOSISDIAGNOSIS

Ewing SarcomaEwing Sarcoma OsteomyelitisOsteomyelitis OsteoblastomaOsteoblastoma Giant cell tumorGiant cell tumor Fibrous DysplasiaFibrous Dysplasia

EWING SARCOMAEWING SARCOMA

DiaphysealDiaphyseal

Onion peel Onion peel

Soft Tissue MassSoft Tissue Mass

OSTEOMYELITISOSTEOMYELITIS

BONE INFARCTBONE INFARCT

CLINICAL PRESENTATIONCLINICAL PRESENTATION

Bone PainBone Pain Most Common Symptom present at Most Common Symptom present at

night or after night or after exerciseexercise

SwellingSwelling Decreased Joint MotionDecreased Joint Motion Pathological FracturePathological Fracture Respiratory symptomsRespiratory symptoms Due to MetastasisDue to Metastasis

CLINICAL SIGNSCLINICAL SIGNS

MassMass Palpable mass may or may not present, Palpable mass may or may not present,

may be warm & tender, firm to hard, increased may be warm & tender, firm to hard, increased skin vascularity over mass.skin vascularity over mass.

Decreased Joint MobilityDecreased Joint Mobility LymphadenopathyLymphadenopathy Respiratory FindingsRespiratory Findings

DIAGNOSTIC TOOLSDIAGNOSTIC TOOLS

Plain X-RayPlain X-Ray Total Body ScanTotal Body Scan MRI of Primary TumorMRI of Primary Tumor CT Scan LungCT Scan Lung BiopsyBiopsy Laboratory StudiesLaboratory Studies

LUNG METASTASISLUNG METASTASIS

CT SCAN

LABORATORY WORKLABORATORY WORK

CBC CountCBC Count LDHLDH Alkaline PhosphateAlkaline Phosphate

INESTIGATIONSINESTIGATIONS

Monitoring ChemotherapyMonitoring Chemotherapy LFTs,RFTs,Electrolytes,UrinalysisLFTs,RFTs,Electrolytes,Urinalysis Echocardiogram(Adriamycin)Echocardiogram(Adriamycin) Audiogram(Cisplatin)Audiogram(Cisplatin)

BIOPSYBIOPSY

Needle BiopsyNeedle Biopsy

craig needlecraig needle

Open BiopsyOpen Biopsy

STAGINGSTAGING

ENNEKING STAGINGENNEKING STAGING

Stage-1Stage-1 Low GradeLow Grade A-Intra CompartmentalA-Intra Compartmental B-Extra CompartmentalB-Extra Compartmental Stage-11Stage-11 High GradeHigh Grade A-Intra Compartmental A-Intra Compartmental B-Extra CompartmentalB-Extra Compartmental Sstage-111Sstage-111 MetastasisMetastasis

TREATMENTTREATMENT

ChemotherapyChemotherapy NeoadjuvantNeoadjuvant AdjuvantAdjuvant SurgerySurgery AmputationAmputation Wide Resection & ReconstructionWide Resection & Reconstruction Resection of Pulmonary Metastasis Resection of Pulmonary Metastasis

Radiotherapy Radiotherapy

CHEMOTHERAPYCHEMOTHERAPY

High dose Methotrexate with leucovorinHigh dose Methotrexate with leucovorin Doxorubicin(Adriamycin)Doxorubicin(Adriamycin) CisplatinCisplatin CarboplatinCarboplatin CyclophosphamideCyclophosphamide IfosfamideIfosfamide

WIDE RESECTIONWIDE RESECTION

Wide excisionWide excision

RECONSTRUCTIONRECONSTRUCTION

Autologous Bone GraftAutologous Bone Graft

AllograftAllograft

ProsthesisProsthesis

RotationplastyRotationplasty

VAN NESS ROTATIONPLASTYVAN NESS ROTATIONPLASTY

VAN NESS ROTATIONPLASTYVAN NESS ROTATIONPLASTY

Van Ness RotationplastyVan Ness Rotationplasty

Amputation ProsthesisAmputation Prosthesis

Total Knee ProsthesisTotal Knee Prosthesis

EXPANDABLE CUSTOM MADE EXPANDABLE CUSTOM MADE PROSTHESISPROSTHESIS

Humeral Prosthesis with AllograftHumeral Prosthesis with Allograft

Vascularized Fibular GraftVascularized Fibular Graft

Vascularized Fibular GraftVascularized Fibular Graft

PROGNOSISPROGNOSIS

5 year Survival ranges from 60% to 85%5 year Survival ranges from 60% to 85% 60 to 65% Treated 5 years ago (2000) will 60 to 65% Treated 5 years ago (2000) will

be alive todaybe alive today

Stage 1 Osteosarcoma has good Stage 1 Osteosarcoma has good prognosis >90% just requires prognosis >90% just requires SurgerySurgery

PROGNOSISPROGNOSIS

Stage 11b Prognosis dependsStage 11b Prognosis depends1.1. Site of Tumor (worse for Axial Skeletal)Site of Tumor (worse for Axial Skeletal)

2.2. Size of Tumor Mass in cm Size of Tumor Mass in cm

3.3. Degree of necrosis from Neoadjuvant Degree of necrosis from Neoadjuvant ChemotherapyChemotherapy

PROGNOSISPROGNOSIS

Stage 111 with Lung Metastasis, Stage 111 with Lung Metastasis, overall Prognosis is 30% depends onoverall Prognosis is 30% depends on

1.1. Resectability of primary tumor& Lung Resectability of primary tumor& Lung NodulesNodules

2.2. Degree of Necrosis of primary TumorDegree of Necrosis of primary Tumor

3.3. Number of MetastasisNumber of Metastasis

4.4. Duration of development of MetastasisDuration of development of Metastasis