giant cell tumors of bone

Giant cell Giant cell tumour of bonetumour of bone

Dr Abdul G SuhailDr Abdul G SuhailMBBS,D.Ortho,MS (Ortho)MBBS,D.Ortho,MS (Ortho)

Assistant Professor in OrthopaedicsAssistant Professor in Orthopaedics

HistoryHistory First described in 1818 by First described in 1818 by

Sir Astley CooperSir Astley Cooper

Described in detail in 1940 Described in detail in 1940 by Jaffe and Litchensteinby Jaffe and Litchenstein

GIANT CELL TUMOURGIANT CELL TUMOUR

DEFINITION:DEFINITION:

DistinctDistinct neoplasm arising from non-bone neoplasm arising from non-bone forming supportive connective tissue of forming supportive connective tissue of marrow with network of stromal cells regularly marrow with network of stromal cells regularly interspersed with giant cells.interspersed with giant cells. ( Jaffe & Liechtenstein( Jaffe & Liechtenstein ) )

AGE OF PRESENTATIONAGE OF PRESENTATION

75-80% OF PATIENTS 20-50 75-80% OF PATIENTS 20-50 YRSYRS

10% 15-20 YRS10% 15-20 YRS

10% >60 yrs10% >60 yrs

<1.7% BELOW 15 YRS<1.7% BELOW 15 YRS

SEXSEX

Male:Female- 1:1.3 (Benign)Male:Female- 1:1.3 (Benign)

-3:1 (Malignant)-3:1 (Malignant)

SITESITE Epiphyseo-metaphysealEpiphyseo-metaphyseal region of long bonesregion of long bones

GCTGCT –Described from all bones –Described from all bones EXCEPTEXCEPT middle ear bonesmiddle ear bones

Axial skeleton- 8%Axial skeleton- 8%

UL:LL-1:3UL:LL-1:3

SiteSite 55% AROUND THE KNEE 55% AROUND THE KNEE

10% in the distal radius10% in the distal radius

6% in the proximal humerus6% in the proximal humerus

SPINE rarely involved (commoner in the SPINE rarely involved (commoner in the sacrum)sacrum)

In the head and neck region the maxilla and In the head and neck region the maxilla and mandible are more commonly involvedmandible are more commonly involved

GIANT CELL TUMOURGIANT CELL TUMOUR

TRANS OSSIOUS EXTENSION- 5%TRANS OSSIOUS EXTENSION- 5%

Common sitesCommon sitesWristWrist

Ankle & footAnkle & foot

ForearmForearm

LegLeg

INTRA ARTICULAR EXTENSION- 10%INTRA ARTICULAR EXTENSION- 10%

Incidence-0.5-5%Incidence-0.5-5% SimultaneousSimultaneous Peculiar featuresPeculiar features

hand hand metaphyseal metaphyseal abundant spindle abundant spindle cellscells

GOLTZ syndromeGOLTZ syndrome `̀

occular defectsoccular defects

skeletal anomaly skeletal anomaly

multifocal GCTmultifocal GCT

MULTICENTRIC INVOLVEMENTMULTICENTRIC INVOLVEMENT

GIANT CELL TUMOURGIANT CELL TUMOUR

SIGNS&SYMPTOMSSIGNS&SYMPTOMS

1.1. PAINPAIN

2.2. SWELLINGSWELLING

3.3. JOINT RESTRICTIONJOINT RESTRICTION

4.4. MUSCLE WASTINGMUSCLE WASTING

5.5. NEUROLOGICAL SIGNSNEUROLOGICAL SIGNS

6.6. PATHOLOGICAL #PATHOLOGICAL #

PathologyPathologyGROSS-GROSS- End of bone is expanded.End of bone is expanded. Eccentric lesion at the epiphyseo-Eccentric lesion at the epiphyseo-

metaphyseal region.metaphyseal region. Thin periosteum.Thin periosteum. Fleshy dark brown, soft, friable mass.Fleshy dark brown, soft, friable mass. Cystic spaces seenCystic spaces seen. .

PATHOLOGYPATHOLOGYGROSSGROSS--

PathologyPathologyMicroscopy-Microscopy- Vascularized network of round,oval or Vascularized network of round,oval or

spindle shaped stromal cells and spindle shaped stromal cells and multinucleated giant cells with numerous multinucleated giant cells with numerous centrally placed nucleicentrally placed nuclei

PATHOLOGYPATHOLOGYMICROSCOPYMICROSCOPY

GradingGradingJaffe,Lichenstein and Jaffe,Lichenstein and

Portis(1940Portis(1940))

GRADE 1-GRADE 1-o Conventional GCTConventional GCTo Stroma is inconspicuous Stroma is inconspicuous o Giant cells dominate the fieldGiant cells dominate the fieldo No atypism of stromal cells and are No atypism of stromal cells and are

loosely arrangedloosely arrangedo Stromal cells are predominantly Stromal cells are predominantly

spindle shapedspindle shaped

GradingGradingGRADE 2-GRADE 2-o Boderline tumoursBoderline tumourso Stromal cells are prominent and tightly Stromal cells are prominent and tightly

packedpackedo Giant cells are less in number compared to Giant cells are less in number compared to

grade 1 and their nuclei may show grade 1 and their nuclei may show atypismatypism

o Stromal cells show atypismStromal cells show atypismo These tumours have a strong chance for These tumours have a strong chance for

recurrence and some may undergorecurrence and some may undergo malignant changemalignant change

GradingGradingGRADE 3-GRADE 3-o Sarcomatous type of stromaSarcomatous type of stromao Frequently metastaseFrequently metastaseo Stromal cells abundant and closely Stromal cells abundant and closely

compacted and present an irregular compacted and present an irregular whorled arrangementwhorled arrangement

o Nuclei are unusually large, irregular and Nuclei are unusually large, irregular and atypicalatypical

o Giant cells are few in number and atypicalGiant cells are few in number and atypical

Modified gradingModified gradingSannerkin et al(1980)Sannerkin et al(1980)

Malignant GCT- Malignant GCT- with frank with frank sarcomatous changes and full sarcomatous changes and full metastatic potentialmetastatic potential

Borderline GCT-Borderline GCT- without sarcomatous without sarcomatous changes but with abnormal mitoses changes but with abnormal mitoses or vascular permeation or bothor vascular permeation or both

Conventional GCT-Conventional GCT- without features without features of any of the above two typesof any of the above two types

No correlation exists No correlation exists between histological between histological grading and clinical grading and clinical behavior of the tumour.behavior of the tumour.

Hence grading not widely Hence grading not widely accepted.accepted.

GIANT CELLS inGIANT CELLS inGiant cell tumorGiant cell tumor Numerous Numerous

nuclei(15-150)nuclei(15-150) Centrally placed Centrally placed

uniform size nucleiuniform size nuclei

TuberculosisTuberculosis Number of nuclei Number of nuclei

are lessare less Peripherally placed Peripherally placed

nucleinuclei

Enneking staging for GCTEnneking staging for GCT

Stage 1-Stage 1-(10-15%)(10-15%)

Patients asymptomaticPatients asymptomatic Discovered incidentallyDiscovered incidentally May cause pathological fractureMay cause pathological fracture Has sclerotic rim on x-ray or CTHas sclerotic rim on x-ray or CT Relatively inactive on bone scansRelatively inactive on bone scans Histologically benignHistologically benign

Enneking staging for GCTEnneking staging for GCT

Stage 2-Stage 2-(70%)(70%)

SymptomaticSymptomatic Often associated with path: fractureOften associated with path: fracture Has expanded cortex but no break throughHas expanded cortex but no break through Is active on bone scansIs active on bone scans Histologically benignHistologically benign

Enneking staging for GCTEnneking staging for GCTStage 3-Stage 3-(10-15%)(10-15%)

SymptomaticSymptomatic Rapidly growing massRapidly growing mass Has cortical perforation with Has cortical perforation with

accompanying soft tissue massaccompanying soft tissue mass Activity on bone scan extends beyond Activity on bone scan extends beyond

the lesion in x raythe lesion in x ray Shows intense hypervascularity on Shows intense hypervascularity on

angiogramangiogram Histologically benignHistologically benign

RADIOLOGYRADIOLOGYType of OsteolysisType of Osteolysis

Geographic destruction (I)Geographic destruction (I)

Moth-eaten (II) Permeative(III)Moth-eaten (II) Permeative(III)

LodwickLodwick

1A1A 1B1B 1C1C

RADIOLOGYRADIOLOGY ExpansileExpansile

RADIOLOGYRADIOLOGY TRABACULATIONTRABACULATION

PURE LYTIC (60%) FINE TRABACULTION(40%)

RADIOLOGYRADIOLOGY

AGGRESSIVENESSAGGRESSIVENESS

LARGE INTRAOSSEOUS CONTENTPURELY LYTIC

CORTICAL BREACHSOFT TISSUE INVASION

SCINTI GRAPHYLess useful

Inconsistent uptake“Doughnut sign”

M.R.I.Soft tissue spread

Joint breachLocate N.V. bundle

C.TIntraossous contentIntra articular spread

Cortical breachSite of window

ANGIO GRAPHYLocate vessels type of feeders

For embolisation

INVESTIGATIONSINVESTIGATIONS

GCT UlnaGCT Ulna

GCT of OlecranonGCT of Olecranon

GCT RadiusGCT Radius

GCT distal femurGCT distal femur

GCT lower end femurGCT lower end femur

GCT FibulaGCT Fibula

GCT CalcaneumGCT Calcaneum

GCT IliumGCT Ilium

GCT C7

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

1.ANEURYSMAL BONE CYST1.ANEURYSMAL BONE CYST

2. GIANT CELL REPARATIVE GRANULOMA2. GIANT CELL REPARATIVE GRANULOMA

3.CHONDROBLASTOMA3.CHONDROBLASTOMA

4.BROWN TUMOR4.BROWN TUMOR

5.INTRA OSSEOUS GANGLION5.INTRA OSSEOUS GANGLION

6.BENIGN FIBROUS HISTEOCYTOMA6.BENIGN FIBROUS HISTEOCYTOMA

Bubbly lesions of boneBubbly lesions of boneTumorsTumors Aneurismal bone cystAneurismal bone cyst Unicameral bone cystUnicameral bone cyst Non ossifying fibromaNon ossifying fibroma OsteoblastomaOsteoblastoma Hyper parathyroidismHyper parathyroidism Chondromyxoid fibromaChondromyxoid fibroma Histiocytosis XHistiocytosis X MyelomaMyeloma Metastasis(kidney,thyroidMetastasis(kidney,thyroid))

InfectionInfection Brodies abscessBrodies abscess CoccidioidomycosisCoccidioidomycosis EcchinococcusEcchinococcus

BIOPSYBIOPSY

CLOSED FINE NEEDLECLOSED FINE NEEDLE

TRUECUTTRUECUT

TREPHINETREPHINE

OPEN INCISIONALOPEN INCISIONAL

EXCISIONEXCISION

TREATMENTTREATMENTSTAGESTAGE TYPETYPE GRADEGRADE SITESITE METASTASISMETASTASIS

STAGE1STAGE1 LATENTLATENTLodwick2Lodwick2

G0G0 T0T0 M0M0

STAGE 2STAGE 2 ACTIVE ACTIVE Lodwick3Lodwick3

G0G0 T0T0 M0M0

STAGE 3STAGE 3 AGGRES.AGGRES. G0G0 T1,2T1,2 M0,1M0,1

SURGICAL TREATMENTSURGICAL TREATMENTStage1& Stage2 --- Intralesional or Marginal Excision

Stage3 --- Wide resection with Reconstruction

Radiation, Embolaisation

Curettage & Bone GraftingCurettage & Bone Grafting

INDICATION STAGE-1&2

ADEQUATE WINDOW

MOTORISED BURR

ExtendedExtended Curettage Curettage

PHENOL

BONE CEMENT

LIQUID NITROGEN

CAUTERY

CO 2 LASER

EN.BLOC EXCISIONEN.BLOC EXCISION

Better result

Dispensable bone ---- Patella, head of Fibula Sub articular lesion

RECONSTRUCTION

Auto graft Allograft Arthrodesis Custom made prosthesis

Reconstruction With Reconstruction With AutograftAutograft

Reconstruction With Reconstruction With AllograftAllograft

Reconstruction With Reconstruction With ArthrodesisArthrodesis

Reconstruction With ProsthesisReconstruction With Prosthesis

MetastasisMetastasis

Benign pulmonary metastasis seen in 2% of patients

unpredictable course

Some spontaneously regress

Others treated by pulmonary wedge resection

25% mortality

Other sites

Lymph nodes, mediastinum ,pelvis.

Pushpavally 32yrs

Pushpavally 32yrs

FEMALE 21YRS.

15-5-92

Lady 28yrs.

Recurrent GCTRecurrent GCT

Recurrent GCTRecurrent GCT

Recurrent GCTRecurrent GCT

Recurrent GCTRecurrent GCT

Recurrent GCTRecurrent GCT

Recurrent GCTRecurrent GCT