BONE TUMORS : GIANT CELL

TUMOR, OSTEOSARCOMA,
EWING SARCOMA
Dr. Vivek k. Gautam
Junior resident
Orthopaedics – SNMC, Agra.
History
• William Enneking : Father of orthopaedic oncology.
• Staged bone tumors :
• Benign
• Malignant
Enniking Classification
Benign Malignant
1. Latent I. Low grade A- intracompartmental
B- Extra compartmental
2. Active II. High grade A- intracompartmental
B- Extra compartmental
3.Aggresive iii. metastasis
GIANT CELL TUMOR
• It is one of the most common bone tumors encountered.
• Though benign tumor, it is locally aggressive and has malignant
potential.
• They have significant bone destruction ,local recurrence and
occasional metastasis
DEFINITION
• Distinct neoplasm arising from non-bone forming supportive
connective tissue of marrow with network of stromal cells regularly
interspersed with giant cells.
• Tumor is called GCT because Giant cells are found.
• These Giant cells resemble osteoclasts…..hence called as
OSTEOCLASTOMA
EPIDEMOLOGY
• Only 5% of PRIMARY bone tumors & 20% of benign bone tumors.
• Almost affects skeletally mature patients in the age group of 15 to 40
with peak incidence in later half of 3rd decade
• Female to male ratio -----1.5 : 1
• But malignant GCT more common in MALES .
SITE OF INVOVLMENT
• Usually SOLITARY lesions 1-2% may
be multi-centric !!
• Seen at
• distal end of femur
• proximal end of tibia
• distal end of radius
• upper end of humerus
• lower end of tibia
• i.e., cancellous disposed bone ends
which are sites of high bone turn over
and osteoclastic activity
• Others like hand , spine and pelvis
CLINICAL FEATURES
SWELLING
• An epiphyseo-metaphyseal , eccentric swelling is seen at the ends of
long bones
• Overlying Skin is stretched & shiny but no engorged veins.
• On palpation, swelling is warm ,tenderness present with bony
consistency
• EGG SHELL CRACKLING may be elicitable when there is too much
thinning of cortex/pathological fracture.
CLINICAL FEATURES
PAIN
• Vague persistent pain at the end of long bones in relation to activity of
the joint
• Pain may increase after a pathological fracture
• Limitation of joint movements due to mechanical block
• Pathological fracture : usually uni-cortical than a complete fracture
• Neurological deficit may be seen in cases involving the spine and
sacrum.
• Metastasis is present in 1-5% cases. most common site being lung..k/a
LUNG IMPLANTS
INVESTIGATIONS
• Blood
• serum calcium
• phosphorus
• ALK.PHOSPHATASE
To rule out hyperparathyroidism
PLAIN RADIOGRAPHS
• Epiphyseo-metaphyseal in location
• Expansible lesion
• Eccentrically situated

• Confined to bone…cortical break-through

indicates more aggressiveness of tumor
• Lysis with or without trabeculations giving rise
to soap bubble appearance
• Geographic distribution rarely extending to
articular cartilage.
• Absence of margin of bone sclerosis or
punctuate calcification
• Absence of intra- lesional bone formation or
ominous periosteal reaction
COMPUTED TOMOGRAPHY
• Confirms the integrity of cortex and outline tumor extent.
• Sub-cortical destruction can be well appreciated.
• Soft tissue extension & relationship to adjacent structures cannot be
studied!!
MRI
• Morphologic analysis & extent of tumor can be assessed.
• Intra-medullary tumors are best seen in T1 weighted images.
• Extra osseous portion is best appreciated on T2 weighted images.
ANGIOGRAPHY
• Not routinely done
• To assess the relationship of major vessels to large tumors
• To know major feeding vessels to tumor
BONE SCAN
• GCT takes up increased uptake of technetium 99.
• Does not correlate to grading or nature of tumor .
• Useful when multi-centric lesions suspected
BIOPSY
• Final diagnostic Tool for GCT
• Types
• FNAC (22 GAUGE NEEDLE)
• CORE NEEDLE BIOPSY (14 GAUGE NEEDLE)
• OPEN INCISIONAL BIOPSY
OPEN INCISIONAL BIOPSY
• Reliable
• Allows pathologists to evaluate cellular morphologic features &
tissue architecture from different sites of lesion
• Use the smallest longitudinal incision
• Use a cautery knife & avoid crushing specimen texture
• Use meticulous heamostasis
PATHOLOGY - GROSS APPEARANCE
• EARLY LESION:
Homogenous ,friable ,reddish
brown mass
• LATE LESION: Variegated
appearance ,blood filled areas
HISTIOGENESIS & MICROSCOPY
• Composed of many multi-
nucleated Giant cells (40-60
nuclei/cell) in a sea of mono-
nuclear stromal cells.
• Stromal cells are the main
neoplastic component of the tumor
which regulate giant cell mediated
bone destruction.
• Nuclei of stromal cells are identical
to that of nuclei of giant cells, a
feature that distinguishes from
other tumors containing giant cells
• Stromal & giant cells in the GCT contain ACID PHOSPHATASE
(TUMOR MARKER) Where as other giant cell variants contains
ALKALINE PHOSPHATSE
• Areas of storiform spindle cell formation, reactive bone formation or
foamy macrophages may be seen
• Secondarily ANEURYSMAL BONE CYSTS may be present
• Indicators of aggressiveness: Increased no. of stromal cells,
hyperchromatism, greater mitotic activity
GRADING OF GIANT CELLTUMOR
CAMPANNCI’S RADIOGRAPHIC ENNEKING STAGING OF GCT
GRADING •
• GRADE I: CYSTIC LESION
• GRADE II: Expansile lytic lesion
with THIN CORTEX but no break
in cortex
• GRADE III: Destructive
radiolucent lesion with cortical
break and soft tissue extension
TREATMENT
PRINICIPLES OF TREATMENT
• The Tumour Is Invasive And Aggressive
• It commonly recurs, may become malignant after unsuccessful
removal.
• Recurrence is treated with en bloc excision.
• En bloc excision is also indicated if the tumour has eroded the cortex
and extended into the soft tissues.
• Eradicate the growth completely at the initial surgery
PRE-OP PLANNING
• Malignancy should be ruled out by prior biopsy and other
investigation.
• OPERATIVE PLAN MUST INCLUDE THIS THREE FACTORS
1.type of resection.
2.The use of adjuvant therapy
3.Type of material to be used to fill the defect
TREATMENT OPTIONS
• SIMPLE CURETTAGE: Intra- lesional curettage ALONE
• EXTENDED CURETTAGE : curettage along with use of adjuvants to
augment curettage EN BLOCK EXCISION IRRADIATION THERAPY
EMBOLISATION BISPHOSPHONATES.
INTRA LESIONAL CURRETAGE
• Adequate exposure with large
cortical window
• High power burr
• Pulsatile jet lavage
• Curettage alone has high
recurrence rate
ADJUVANTS TO CURRETAGE
• Advantage of using adjuvants
• It eliminate the microscopic disease and reduces recurrence
• Curettage and cementation causes a 2mm osteolytic lesion zone
surrounding the cement due to thermal injury
• PHENOL-12-50% conc
• Easily absorbed
• Nephrotoxic
• Soft tissue complication
• HYDROGEN PEROXIDE
ADJUVANTS TO CURRETAGE
PMMA BONE CEMENT -
Bone cement +ADRIAMYCIN+METHOTREXATE to reduce recurrences
PRINICIPLE: heat of polymerization or direct toxicity of monomer
CRYOSURGERY WITH LIQUID NITROGEN
it create 1-2cm zone of tissue necrosis.
Local complications…thermal shock, dehydration, wound healing
problems.
Not easily available & costly
Storage difficulties
RECONSTRUCTION OF RESIDUAL DEFECT
• Bone grafting
autogenous bone graft
Allograft
artificial bone graft substitutes
demineralised bone matrix
• Bone cement
ADVANTAGE DRAWBACK

Remodelling along stress lines Auto graft quantity is less

Reconstruction is permanent Donor site morbidity

Restores bone stock Allograft is expensive.. equires bone bank

Restores normal biomechanics at joint Recurrence is difficult to distinguish from graft

surface(theortical) resorption BONE GRAFT
PMMA BONE CEMENT
ADVANTAGE DISADVANTAGE

Immediate structural support and early Not a biological material

ambulation

MMA monomer is cytotoxic Though strong in compression but weak when

subjected to shear and torsional forces

Thermal effect Radiographic detection of Fear of long term degeneration of articular

recurrence is easier cartilage in sub-chondral lesion in wt bearing
stress
SANDWITCH TECHNIQUE
• When tumor is <1cm from articular surface,
• The incidence of degenerative changes in cartilage after the use of
cement alone is 2.5 times greater than when tumor is >1cm away
• In such conditions, multilayer reconstruction technique is
recommended
• Interposing bone graft between the cartilage &cement reduces heat
damage and resultant degenerative changes
Sandwich technique
•
A layer of gel foam is layered over cement to reduce heat damage to
graft.
EN BLOC RESECTION AND
SUBSEQUENT
RECONSTRUCTION/ARTHODESIS
• Initial procedure of choice in more aggressive tumors .
• 2 cm of normal tissue is also excised.
• Defects are filled with cancellous bone grafts, freeze dried allografts
or prosthesis.
• This technique has low recurrence rate
Resection :Indications
stage 3 lesions
cortex destroyed and soft tissue extension present
Recurrences
Large defects are expected
Joint surface destroyed or cannot be salvaged
Certain bones which can be sacrificed such as
• Lower end of ulna
• Upper end of fibula
RECONSTRUCTION OPTIONS

BIOLOGIC
• AUTOGRAFT ARTHRODESIS
• LIVE MICROVASCULAR FIBULAR GRAFT
• OSTEO-ARTICULAR ALLOGRAFTS
• ILIZAROV METHOD
ENDOPROSTHETIC JOINT REPLACEMENT
• eg: custom mega prosthesis
EMBOLISATION
• Trans-catheter Embolisation of blood supply of Certain un-resectable
tumors like sacrum & pelvic

PREOP EMBOLIZATION also

Brings down size of tumor & provides pain relief
Re-embolisation needed at monthly intervals
AMPUTATION
• Malignant tumour
• Fungation
• Recurrence after surgery and irradiation
• Deep seated associated infection
• Extensive destruction of bone
• Severe disability
RADIOTHERAPY
• When complete excision or curettage is not possible
• Aggressive, multiple/ recurrent tumor
• Lesions of spine & sacrum
• The recommended dosage is 1,500 to 5,000 rads for 5 to 6 weeks
using mega voltage therapy cobalt 62
• It may induces malignant change if it is given to the benign lesion.
BISPHOSPHONATES
• Pamidronte / zoledronate can be given
• They target osteoclast like giant cells.
• Limit tumor progression
OSTEOSARCOMA (OSTEOGENIC SAR
COMA)
• Highly malignant primary bone tumour
PATHOLOGY
• Malignant tumor of mesenchymal cells.
• Osteoid or bone formation by tumor cells
CLASSIFICATION
• Based on clinical setting:
a) Primary osteosarcoma
 15-25 years
No known pre-malignant conditions
b) Secondary osteosarcoma
Older age(>45yrs)
Paget’s disease, fibrous dysplasia…
• Based on dominant histo- morphology:
a) Osteoblastic
b) Chondroid
c) Fibroblastic
d) Telengiectatic or osteolytic type
FEATURES
• Age of onset: – 15-25yrs
• Sites of origin:
– Any bone
– Lower end of femur
– Upper end of tibia
– Upper end of humerus
Gross appearance
Osteoblastic tumour: Greyish white, hard and gritty feeling when cut.
Chondroid type: Opalescent and bluish grey.
Fibroblastic: Typical fish flesh sarcomatous appearance.
Telengiectatic: Tumour necrosis and blood filled space
Histologically
• Basically anaplastic mesenchymal parenchyma with tumour cells
surrounded by osteoid.
Clinical features
• Pain – constant and boring
• Swelling
• Pathological fracture
EXAMINATION
• Swelling in the region of metaphysis.
• Skin over the swelling: Shiny with prominent veins.
• Swelling warm and tender.
• Margins: Not well defined.
• Mechanical block of the swelling.
• Compression
• Regional lymph node enlargement, usually reactive
INVESTIGATIONS
• RADIOLOGICAL EXAMINATION:
• Area of irregular destruction in the metaphysis. Cortex overlying
lesion eroded. – New bone formation in the matrix.
• Irregular periosteal reaction
• Codman’s triangle: – Triangular area of subperiosteal new bone – At
tumour-host junction at tumour end.
• Sun-ray appearance:
– Tumour grows to overlying soft tissues.
– New bone laid down along the blood vessels within the tumour
growing centrifugally.
SERUM ALKALINE PHOSPATASE (SAP)
Generally elevated
– No diagnostic significance.
– Useful for follow-up
– Rise after tumor removal
- Indicator of recurrence or metastasis.
BIOPSY
• open biopsy
TREATMENT
• Aims:
– Confirm diagnosis
– Evaluate spread
– Adequate treatment
Confirmation of diagnosis
– Histologically, Tumour new bone formation- pathognomonic of
osteosarcoma.
– Or Clinical and radiological picture
• Evaluation of spread of tumour:
– Lung-Earliest site.
– Chest X-ray.
– CT scan
• Extent of involvement need to be known to:
– Plan amputation surgery
– Plan limb saving operation
Treatment of the tumour:
– Local control:
• Surgical ablation.
• Amputation remains the mainstay.
• ROLE OF RADIOTHERAPY:
– Surgically inaccessible sites.
Control of distant macro or micro-metastasis
• ROLE OF CHEMOTHERAPY:
– Drug used: high dose Methotrexate, Endoxan…
• ROLE OF IMMUNOTHERAPY
FOLLOW UP
• Patient is checked up every 6-8 weeks.
• Recurrence needs to be diagnosed early and treated.
PROGNOSIS
• Without treatment : death within 2 years.
• 5 year survival with surgery alone(20%).
• Primarily lytic type(telengiectatic) osteosarcoma-worst prognosis
 Ewings sarcoma
• Introduction
Identified by james Ewing.
2nd most common tumor in children.
• Epidemiology
• Occurs most commonly in 2nd decade
 80% occurs between ages 5 and 25

• M: F
 1.3 <10yrs
 1.6:1>10yrs
Routes of Spread
• Direct Extension- into adjacent bone or soft tissue.
• Metastasis generally spread through blood stream.
• Nearly all patients have micromets at diagnosis, so all need chemo.

CYTOGENETICS
• t(11;22) , t(24;12) presents in 90-95% .
• C-myc protooncogene is frequently expressed in Ewing’s.
• PAS +ve.
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