DR ASHWANI PANCHAL

JSS MEDICAL COLLEGE

MYSORE

● aggressiveness of these lesions is between

purely benign and frankly malignant.● Today in this seminar brief dicussion of such benign

aggressive tumors would be done

● GIANT CELL TUMOR

● CHONDROBLASTOMA

● OSTEOBLASTOMA

● CHONDROMYXOID FIBROMA

● LANGERHANS CYST HISTOCYTOSIS

● Sir Astley Cooper first describe in 1818 emphasizing its

benign nature.

● Dr Paget called it “brown or Myeloid tumour” in 1853.

● WHO- ‘’An aggressive , potentially malignant lesion’’

Its an aggressive lesion characterised by well- vascularised

tissue composed of spindle cells and multinucleated giant cells

uniformly dispersed throughout the tumour.

● acc to mayo clinic series 5% of neoplasms

of bone.

● common in some parts of andhra pradesh in

india.

● Usually after epiphyseal closure.

● Hence age group involved is 20 to 40

years(70%) with slight female predominance

(3:2 ).

● Gradual Decrease in after fifth decade.

Site of Involvement

Situated mostly at epiphyseo-metaphyseal region of long bones.

AROUND KNEE JOINT 55 %

DISTAL END OF RADIUS 10-12 %

UPPER END OF HUMERUS 7%

LOWER END OF TIBIA 3-4 %

SMALL BONES OF HAND AND FEET 2%

SPINE ,SKULL AND PELVIS 15-17%

● complains of vague persistent pain over the

swelling at end of long bones…swelling

present from few weeks to several months.

● Note- This pain could have increased after

pathological fracture which may bring the

patient to surgeon for first time.

Overlying skin is stretched , shiny

with no engorged veins.

● PALPATION- The swelling is

warm , smooth with variable consistency, predominantly

bony and tenderness is present on firm palpation.

● EGG SHELL CRACKLING-ELICITABLE WHEN THERE

IS PATHOLOGICAL FRACTURE OR TOO MUCH

THINNING OF CORTEX.

ROM at adjacent joint can be restricted due to

mechanical block.

Plain Radiograph-

● Tumour will show large , sharply

circumscribed area of reduced density

asymmetrically located in the epiphysis,

begining subcortically and extending

towards metaphysis.

● SOAP BUBBLE APPEARANCE-

multilocular and trabeculated appearance of

tumor.

● Geographical destruction type of osteolysis.

● cortical thinning.

● no sclerotic rim and new bone formation.

● Helps in confirming the integrity

of cortex and outlining the

tumour extent.

● Subcortical destruction can

be well appreciated with a

ct scan.

● Limitation- soft tissue

extension of tumour and its

relationship with adjacent

structures cannot be seen .

● With mri the morphologic analysis

and extent of disease into

surrounding soft tissue can be

assessed .

● As in CT SCAN, sub cortical

destruction can be well appreciated

by multiplanar mri.

● Intramedullary tumors best

appreciated in T1 weighted images.

● Extraosseous Tumors best

appreciated in T2 weighted images.

● to check Relationship of major

vessles to large tumors.

● Does not correlate with

grading of tumor.

● GCT takes up increased uptake of technetium

99.

● Again does not have any correlation with

grading of tumor.

This is the final diagnostic tool for diagnosis of gct.

Sample can be taken by

● OPEN INCISIONAL BIOPSY

● FINE NEEDLE ASPIRATION

● CORE NEEDLE BIOPSY

● Epiphyseal end of long bone will be expanded with

thining of periosteum and cortex, being easily broken

by handling.

● Composed of ragged , very friable, readily bleeding

tissue containing variously sized cavitations and

small cysts.

● colour -varies from reddish brown to chocolate color

in which vascular tissue predominates , to greyish or

mottled where connective tissue is major component.

● no evidence of periosteal new bone formation.

● the inner wall of tumor is lined by a fibrous capsule

from which the septae extend inwards to partition the

tumor.

● Multinucleated giant cells and spindle

cells are the main component .

● GIANT CELLS in this tumor are

characteristic and specific having size

of 10 to 100 microns with centrally

placed uniform sized nuclei

numbering 15 to 150 max.

● SPINDLE CELLS- They are oval

,elongated and contain relatively

large chromatinized nucleus and

small acidophilic cytoplasm

● The appearance of spindle cells indicates the malignant

potential of tumor.

POINT TO BE NOTED-

● Appearance of giant cells is not diagnostic. Giant cells are

also seen in lesions like aneurysmal, unicameral cyst, non

ossifying fibroma, chondroblastoma and brown tumor of

hyperparathyroidism

● appearance of spindle cells is important.(main neoplastic

component)

● Grade 1- tumor has well

marinated border of a thin rim

of mature bone, and the

cortex is intact or slightly

thinned but not deformed.

● Grade 2- tumor has relatively

well defined margins, but no

radio opaque rim, cortex is thin

and expanded but is present.

Grade 3- cortex is perforated

with extension of tumor into soft

tissue.

Based on clinical radiological and histopathological features

Stage 1- LATENT(10 - 15%)

● Patient is asymptomatic, discovered incidentally.

● May be associated with pathological fractures.

● Radiologically-tumor is intracapsular, with well defined margins and sclerotic rim.

no cortical destruction.

Stage 2- ACTIVE ( 70-75 %)

● Patient is symptomatic.

● Often associated with pathological fracture.

● Radiologically- intracapsular, has expanded or thinned out cortex ,but there is no

breakthrough the cortex.

Stage 3 (aggressive) : 10 - 15 %

● Symptomatic

● Rapidly growing mass

● Radiologically- Extracapsular, has cortical

perforation

● Will show intense vascularity on angioram

Note-

TNM Classification system is not applicable to

GCT because anatomically it remains

intracompartmental for a long time within well

formed capsule of the periosteum and soft

tissue.

1. Aneurysmal bone cyst

2. Brown tumor of hyperparathyroidism

3. non ossifying fibroma

4. Unicameral bone cyst

5. Fibrous dysplasia

6. Chondroblastoma

7. Giant cell reparative granuloma

8. ossifying fibroma

9. osteogenic sarcoma

● Majority of tumors are benign, have 30 to 40

% recurrence and has tendency to

metastasize.

● To eradicate the growth completely at initial operation

● to avoid ablation of limb

● to maintain possible function

● Traditionally

: intralesional curettage/resection with bone graft

:recurrence 35-42 %

● HISTORICALLY TREATMENT CONSISTED OF SIMPLE CURETTAGE

● BUT RECURRENCE RATES > 50%

● FOR DEFECTS AFTER RESECTION OR CURETTAGE,EITHER

ALLOGRAFT OR BONE CEMENT USED AS FILLING AGENTS

EXTENDED CURETTAGE –USE OF A POWER BURR TO ENLARGE THE

CAVITY 1-2 CM IN ALL DIRECTIONS IS NOW CONSIDERED STANDARD

ADJUNCT Tx

●PMMA, Liquid N2, Phenol, l,electrocautery.

–Local extension of margin

–Kill residual foci and remaining tumour cell

● ASSOCIATED WITH PATHOLOGIC

FRACTURES & WOUND HEALING PROBLEMS

ADVANTAGE:

• RESTORING NORMAL BIOMECHANICS TO JOINT

SURFACE

•PREVENT FUTURE DEGENERATIVE JOINT DISEASES

•RESTORING BONE STOCK

•DISADVANTAGES

: JOINT MUST BE PROTECTED FOR AN EXTENDED

PERIOD OF TIME TO PREVENT A PATHOLOGICAL

FRACTURES.

● TUMOUR RECURRENCE IS DIFFICULT TO

DISTINGUISH FROM GRAFT RESORPTION.

•THE ABOVE DISADVANTAGES OVERCOME BY USE OF BONE

CEMENT

•PROVIDES IMMEDIATE STABILITY-HENCE QUICKER

REHABILITATION

•EASIER DETECTION OF RECURRENCE SEEN AS EXPANDING

RADIOLUCENCY ADJ TO CEMENT

•KILLS RESIDUAL TMR CELLS THROUGH POLYMERISATION HEAT.

● CYTOTOXIC AGENTS- METHOTREXATE AND ADRIAMYCIN CAN BE

INCORPORATED IN BONE CEMENT.

.

Curettage with bone

cementing

•INITIAL PROCEDURE OF CHOICE AND HERE 2 CM OF NORMAL

BONE IS ALSO EXCISED

•

•DEFECTS ARE FILLED WITH CANCELLOUS BONE

GRAFTS,FREEZE DRIED ALLOGRAFT OR PROSTHESIS

resection of tumor in lesions of

sacrificable part

● lower end ulna

● upper end of fibula

● phalanges

● metatarsal rays

RECURRENCE IS ALMOST NILL

Resection and

reconstruction with

Autograft

•AROUND THE KNEE,A HEMI CONDYLAR OSTEOARTICULAR

ALLOGRAFT RECONSTRUCTION OR A ROTATING HINGE

ENDOPROSTHESIS MAY BE NECESSARY

•FOR AGGRESSIVE LESION OF DISTAL RADIUS,PRIMARY

RESECTION AND RECONSTRUCTION WITH A PROXIMAL FIBULAR

AUTOGRAFT INDICATED

•FOR GCT AFFECTING LOWER END OF FEMUR OR UPPER END

OF TIBIA

• AFTER EN BLOCK EXCISION RECONSTRUCTION CAN BE

DONE BY

1.TURN-O-PLASTY TECHNIQUE

2.ARTHRODESIS

3.ARTHROPLASTY

RADIOTHERAPY- Disappointing recurrence rate of 50-70%.

● malignant sarcomatous transformation ocuured in 7-10 %

cases

CHEMOTHERAPY- NO EFFECTIVE CHEMOTHERAPEUTIC AGENTS

AVAILAIBLE FOR MANAGEMENT OF GCT.

● Most local recurrences and pulmonary metastases occur within 3

years or even upto 20 years.

● Patient should have radiograph of primary tumor site and of the

chest at

>> 3 MONTHS INTERVAL FOR 1YR

>> 6 MONTHS INTERVAL FOR NEXT 2 YRS

>>AND ANNUALLY THEREAFTER

•treatment is same as for primary lesions.

•after biopsy shows that tumor is still

benign,repeat curretage or resection is

performed.

● Codman in 1931 first described..”Codmans

tumor”

● It is a rare , benign bone tumor of immature

cartilage cells derivation arising in epiphysis

consisting of polygonal chondroblast, foci of

chondroid, osteoclast like giant cells and

small foci of calcifications.

● INCIDENCE- less than 1 % of primary bone

tumors.

● AGE

Ranges - 3 to 73 years.

Usually teenagers, before obliteration of epiphyseal

plate.

90% Reported in 5 to 25 years.

● SEX

Male:female 2:1

● SITE

Epiphyseal region of long bone, occasionally extends to adjacent

metaphysis.

● COMMON OCCURRENCE

lower extremity(72%)...in which 50 % cases have been reported

knee

Proximal humerus 18%

Proximal Tibia 17 %

Distal Femur 16 %

Proximal femur 16%

Ankle bones 9%

SIZE - 1 to 6 cm

CLINICAL FEATURES-.

>Pain & swelling – several months.

>May be referred to nearest joint

>Some loss of joint function & muscle wasting.

>Joint effusion esp. around knee.

>Pathological fractures – rare.

X – RAYS:

>Lytic area –

>Oval or round

< ½ of epiphyseal area.

>Thin rim of sclerosis

>Punctate or streaky calcification.

● In metaphyseal extension,

i.e tumor crossing growth

plate results in –

Eccentric location &

bulging expansion of

cortex

Gross:

● Usually well demarcated

lesions.

● Capsule – thin, easily

disruptable

● Cut section- Soft, reddish –

purple, friable, focally fritty

tissue.

● Cystic spaces and

haemorrhages may be seen.

MICROSCOPIC:-

● Islands of chondroblasts

with uniform polyhedral

closely packed cells.

● Background of fibrous

stroma cells within the

islands – PAVING

STONE APPEARANCE.

● Cells – round, plump and active little

immature matrix is present.

● Pericellular lattice-like fine

calcification – “CHICKEN WIRE” or

“PICKET FENCE” pattern are seen.

● Small granular purplish areas of

microcalcification.

● Multinucleated Giant cells –

scattered in stroma.

● Often prominent, dilated blood

vessels at centre & periphery are

present.

● Enchondroma

Hand, diaphysis.

● G.C.T.

Eccentrical, soap bubble or trabecular pattern

No calcifications

Often after closure of growth plate.

● Central chondrosarcoma

Slow growth, severe pain & margins not demarcated on xrays.

● Chondromyxoid fibroma

Site- Metadiaphysis

Septae are present.

● Curettage and autologous bone grafting – high recurrence rate (10 –

35%).

● Close follow up & observation for all till skeletal maturity – in

patients , whose potential growth is remaining and lesions abuts the

epiphyseal plate, lesions should be followed up and observed, instead

of premature or aggressive treatment

● Marginal extra capsular excision – when growth plate not at all at

risk, curettage and excision is done.

Defect can be obliterated with Autogenous bone graft.

● Methacrylate adjunct –

when excision impractical & intracapsular curettage

is of high risk of recurrence due to surgical

inaccessibility.

● Curettage followed by cryosurgery:

>In case of recurrence or when associated with ABC.

>Yields consistent good results with a high cure rate when

entire tumor is adequately frozen by liquid nitrogen.

Radiosensitivity:

● Radiosensitive tumor

● Not used for uncomplicated cases – potential hazards

of irradiation induced malignant transformation.

● Jaffe and lichtenstein first described it in 1956.

● Rare bone tumor,

● 1 % of all primary bone tumors

● 3 % of benign bone tumors.

It is a Solitary benign progressively growing bone tumor

histologically similar to osteoid osteoma , size, clinical and

radiological features differentiate it.

AGE

● Young person 10 to 30 years (80%)

SEX

● Males: female -3:1.

SITE

Vertebral Column 34 %

Extremities 30%

Hands and feet 14%

Skull and facial bones 15 %

● In long bones it occurs in medullary portion of metaphysis.

Rarely juxtacortical or periosteal osteoblastomas are

reported.

● In Spine- spinous and transverse process are frequently

involved. lesions in body are rare.

● Pain- Dull , aching, persistent and localized.

● Slight local tenderness and palpable swelling of increasing

size.

● spinal location

> radiculopathy(50%).

>rarely produce neurological symptoms .

>scoliosis and muscle spasm may appear.

● lesions of extremities may present with wasting and limp.

● lesion is well circumscribed, radiolucent,

eccentric and with an intact surrounding shell

of bone .

● centrally there is lytic area >2cm with

surrounding sclerosis.

● In vertebrae it is seen as

definitely expansile radiolucent

growth containing granular

radiopacities

● ct scans are better appreciated

for vertebral lesions

.

● it can show cotton wool

appearance when

calcification of tumor

tissue has occured...if

cotton wool appearance is

seen in lesion...it is

diagnostic.

GROSS

● well circumscribed, 2-10 cm.

● surrounded by shell of cortical bone or thickened

periosteum.

● Haemorrhagic, granular , friable and calcified

lesions.

•Vascular spindle-cell

stroma with abundant

irregular spicules of

mineralized bone and

Osteoid

OSTEOBLASTOMA OSTEOID OSTEOMA

1. INCIDENCE one fifth as common as

osteoid osteoma

10% of benign bone tumor

2. COMMONEST LOCATION Vertebral column, often

posterior element

Proximal femur

3.CLINICAL PRESENTATION Pain inconsistent pain persistent , nocturnal

4. RADIOGRAPHY Size > 2 cm

no or minimal perifocal

osseous reaction

size < 2cm, perifocal

osseous reaction is

marked.

5.HISTOLOGY

>Osteoid trabeculae with

discontinuous and irregular

bone formation,

> abundant fibrous stromal

>osteoid trabaculae with

continuous and regular

bone formation.

>Scanty stromal reaction

● OSTEOSARCOMA

No ALP rise in osteoblastoma, stromal cells are not large, but

are relatively uniform.

● GCT

>>more than half are located near knee, or distal radius.

>>soap bubble appearance.

● Curettage and resection.

● bone grafting of defect.

● In spine- if resection causes instability,

instrumented fusion can be necessary.

● Radiation- some authors have recommended,

adjuvant radiation therapy for spinal lesions, as

revision surgery is difficult.

● Rare tumour, least common benign cartilaginous tumors.

● Can be misdiagnosed as chondrosarcoma...as it is

composed of similar cytological features, however

radiological and clinical findings are of benign tumor .

AGE

● 2nd and 3rd decade, rare in children.

SEX

● No predominance.

LOCATION

● metaphyseal region of large tubular

bones.(eccentric)

● can also involve thin tubular bones- fibula,

phalanx and calcaneus.(usually entire width)

CLINICAL PICTURE

● In young adults tumor causes mild or no pain,

slowly increasing local swelling and palpable

tender tumor.

● Rarely metastasizes

● In less than 10 years age group- symptoms

are more pronounced.

RADIOLOGICAL FINDINGS

XRAY

● in long bones

>>Lesion -translucent mass of variable

sizes , located eccentrically in metaphysis

>> on medullary aspect of lesion- margin

is scalloped and sclerosed.

>>Cortex - Expanded and thinned out,

may appear interrupted.

● in case narrow tubular bone

(fibula) or small tubular bone

(phalanx)- generally entire

width of bone is involved

● fusiform expansion and

thinning of both cortices.

PATHOLOGY GROSS

● Outer surface- covered by thin shell of newly

formed periosteal bone.

● Cut Surface-solid tumor mass of greyish white

or bluish grey color,resembles cartilage and

sometimes contain cavities of mucoid tissue

● consistency - usually firm.

● Calcified areas are unusual.

● no bony septa traverse the tumor.

MICROSCOPIC

•Tumor is composed of lobulated

and pseudo lobulated areas of

stellate cells with indistinct

cytoplasmic borders,lying within

the central portion of lobule and

widely separated by mucin like

material.

● Curettage is not sufficient, as tumor may recur.

● En Bloc excision and filling cavity with

autogenous bone results in high rate of cure.

● Despite Wide excision , if tumor recurs then

additional studies should be done to rule out

malignant transformation.

Curettage and Bone cementing

● Previously called HISTIOCYTOSIS X

● Characterised by widespread dissemination of

reticuloendothelial system.

● Syndrome constitutes following clinical conditions

1. Eosinophilic Granuloma

2. Hand -Schuller -Christian disease

3. Letterer -Siwe disease.

Orthopaedic surgeon is primarily concerned

with

EOSINOPHILIC GRANULOMA OF BONE

● Self limited benign bone destructive lesion characterised by histocytic

and eosinophilic leucocyte infiltration.

Incidence

● Usually in first two decades with peak incidence in 5 to 10 years

group.

● solitary lesion usually seen in ribs vertebrae, skull, flat bones and long

tubular bones.

● multiple lesions are rare - ocurrance in skull and femur

● diaphysis and metaphysis are commonly involved.

● Acute onset

● complains of dull aching pain in a limited local area of bone.

● local tenderness , warmth and swelling over the affected bone.

● pathological fracture of long bone or vertebral collabs can be

seen.

● Disease usually progress for few weeks to few months , and

recovery is usually spontaneous

● Rapidly destructive lytic process

producing punched out

appearance

● In diaphysis of long bones lesions may

have aggressive permeative appearance

with periosteal reactive bone formation.

● Eosinophilic granuloma of

vertebral body

● showing marked flattening of

vertebral body or vertebral

plana

•Diagnosis is made by identification of

langerhans cell.

•Mixture of eosinophils, plasma cells,

histiocytes along with peculiar large

mononuclear giant cells (Langerhans cells)

with abundant pale cytoplasm and cleaved

nuclei.

•Necrosis, fibrosis and reactive cells are

evident

● Microscopically diagnosis is made by identification of

langerhans cell.

● Recommended treatments incd-

>> corticosteroid infiltrations

>> radiation therapy(300 to 600 rds)

>> Curettage with or without bone grafting

.

• Chronic form of histocytosis

• There is wide disseminated infiltration of reticulo

endothelial system by histiocytes and eosinophilss.

• INCIDENCE

• Disease begins in childhood (5-10years) and continues to

adulthood.

• Commonly involves membranous bones like skull and

organs like pituitary , hypothalamus lungs, spleen and

liver.

RADIOLOGICAL FINDINGS

• very large well demarcated defects in skull .

• In long tubular bones, presence of lytic defects without

surrounding zone of sclerosis.

• PATHOLOGY

• There is replacement of bone and tissue by infiltrating

granulomatous tissue

• In orbit , progressive infiltration causes exophthalmos.

• Involvement of posterior pituitary and hypothalmos leads

to diabetes insipidus

PROGNOSIS

• Overall mortality reported in 30 %.

• depends upon number of organ involved and quantity of

dissemination

• TREATMENT

• For accessible lesions , curettage and bone grafting or

segmental resection is preffered.

• Low dosage radiotherapy is also advocated.

• For widespread dissemination, combination of

chemotherapy, steroid and radiotherapy are considered.

• It is an acute fulminating generalized disorder affecting

reticulo endothelial system and again characterised by

histiocytic infiltration.

• disease involves young age groups (1 – 3 years)-

• most common site- skull , ribs and vertebrae.

• organs involved – lymph nodes, tonsils, thymus, spleen,

liver, lungs , heart, pancreas, renal pelvis and endocrine

glands.

Clinical features

• Acute onset

• Patient febrile with lymphadenitis ,

hepatosplenomegaly, dermatitis eruption, destructive

lesions in skull, vertebrae .

• Bacterial infections of upper GI and respiratory tracts

is common

TREATMENT

• involves use of appropriate antibiotics, radiation and

steroids.

• Chemotherapeutic drugs are also tried.

• course of disease is very fatal.

1. ORTHOPAEDICS PRINCIPLES AND THEIr APPLICATION:

SAMUEL L TUREkS

2. CAMPBELLS OPERATIVE ORTHOPAEDICS

3. TEXTBOOK OF ORTHOPAEDICS AND TRAUMA - GS

KULKARNI

4. MERCERS ORTHOPAEDIC SURGERY

5. DAHLINS BONE TUMORS