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Chondrosarcoma 1

Chondrosarcoma is the third most common primary bone malignancy. It is a malignant tumor arising from cartilage producing cells. Radiographs often show characteristic punctate, popcorn-like calcifications. High grade lesions are associated with bone destruction and soft tissue extension. Biopsy is only recommended for diagnostic uncertainty. Treatment involves wide surgical excision with clear margins. Curettage may be considered for low grade tumors in non-weight bearing bones. Prognosis depends on grade, size, and location, with larger, high grade, or pelvic tumors carrying a worse prognosis. Long term follow up is important due to risk of late recurrence.

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0% found this document useful (0 votes)
174 views74 pages

Chondrosarcoma 1

Chondrosarcoma is the third most common primary bone malignancy. It is a malignant tumor arising from cartilage producing cells. Radiographs often show characteristic punctate, popcorn-like calcifications. High grade lesions are associated with bone destruction and soft tissue extension. Biopsy is only recommended for diagnostic uncertainty. Treatment involves wide surgical excision with clear margins. Curettage may be considered for low grade tumors in non-weight bearing bones. Prognosis depends on grade, size, and location, with larger, high grade, or pelvic tumors carrying a worse prognosis. Long term follow up is important due to risk of late recurrence.

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CHONDROSARCOMA

DR. KUMAR SUPRASHANT


DNB RESIDENT
HINDU RAO HOSPITAL AND
NDMC MEDICAL COLLEGE
CHONDROSARCOMA
OVERVIEW
1.Defination

2.Frequency

3.Types
Defination c/f
Frequency radiology
Epidemiology histology
Etiology

4.Treatment
CHONDROSARCOMA
DEFINATION
Malignant tumour of cartilage producing cells
CHONDROSARCOMA
FREQUENCY
9% of primary malignancies of bone

3rd most common primary malignancy of bone


after multiple myeloma and osteosarcoma

2nd most common non hematological primary


malignancy of bone
CHONDROSARCOMA
TYPES
Primary chondrosarcoma
Secondary chondrosarcoma
Periosteal chondrosarcoma
Dedifferentiated chonrosarcoma
Clear cell chondrosarcoma
Mesnchymal chondrosarcoma
Primary chondrosarcoma
Malignant cartilage tumour arising in
previously normal bone
a/k/a central or conventional chondrosarcoma
90% of all chondrosarcoma
Age group- 40 to 60 yrs
Male: female = 3:2
Sites
Pelvis most common site(ilium mc bone)
Proximal femur
Proximal humerus
Ribs
(although rarely occour in hand but mc
primary malignancy of bone in hand)
Clinical feature
Pain
Palpable mass
Asymptomatic incidental finding
Pathological fracture( in high grade
chondrosarcoma)
Radiographic findings
Frequently diagnostic
Arising in medullary cavity with irregular
matrix calcification- punctate , popcorn,
comma shaped calcification( rings and arcs
appearance)
Bone destruction, cortical erosions,
periosteal reaction, soft tissue mass
Chondrosarcoma from
iliac bone with soft
tissue extention
Axial CT image showing mass
arising from iliac bone with
chondroid calcification which
suggest chondrosarcoma
RING AND ARC CALCIFICATION
Differenciating encondroma from
chondrosrcoma

Enchondroma and low grade chondrosarcoma


may appear radiologically similar

Similarity- both located in metaphysis with


stippled calcification and endosteal scalloping
Histology similar in enchondroma and low
grade chondrosarcoma
Difference
Endosteal scalloping of more than 2/3rd of cortical
thicness- chondrosarcoma

Aggressive changes such as cortical erosion , bone


destruction, periosteal reaction, soft tissue
mass- chondrosarcoma

size> 5 cm in axial skeleton- predictor of


malignancy

Apperance of lysis in previously calcified area-


malignancy
Site- aggressive lesion in hand still considered
benign whereas same amount of aggressive
behaviour in pelvis or proximal femur is
dignostic of chondrosarcoma
CHONDROSARCOMA ENCHONDROMA
CT scan
Differentiate benign from malignant lesion
For characterizatiion of lesion in anatomically
complex areas- sacrum, pelvis
MRI
Depict high water content of lesion with
lobulation at margins
Best to see intramedullary extent
Cotical erosion, bone destruction,
reactionary edema, soft tissue extention
are well depicted
Intramedullary
extention & soft
tissue extention
Bone scan
Radionucleotide uptake has been graded
GRADE 1- uptake less than ASIS
GRADE 2- uptake same as ASIS
GRADE 3- uptake more than ASIS
Grade 3 uptake is consistent with chondrosarcoma
than enchondroma
PET scan
Standard uptake value (suv) may be helpful in
diffrentiating benign from malignant
catrilaginous lesion
Histology
Composed of malignant cells with abundant
cartilaginous matrix, cartilage permeates the
host bony trabeculae
Histologically three grades
Grade 1- less cellular and less nuclear atypia
(vis--vis enchondroma)
Grade 2 - cellularity and nuclear atypia
Grade 3- cellularity and pleomorphic
anaplastic cells
Factors which favour malignancy
Hypercellularity, plump nuclei, binucleate cells,
permeative pattern, entrapment of bony
trabeculae

Overall anatomical location, clinical


behavior, radiologic finding must be
considered along with histology to
diffrentiate enchondroma from low
grade chondrosarcoma
Intramedullary ,soft
tiisue extention of tumour
with calcification
SECONDARY CHONDROSARCOMA
Arise at the site of preexisting benign cartilage
lesion
Ollier disease(multiple enchondroma)
Maffucci disease( multiple enchodroma+ soft
tissue hemagioma)
Solitary osteochondroma(1%)
Multiple heriditary exostosis(5%)
Synovial chondromatosis
Chondromyxoid fibroma
Periosteal chondroma
Chondroblastoma
Fibrous dysplasia
Previous irradiation
Chondrosarcoma developing on the surface of
bone as a result of malignant transformation
of cartilage cap of preexisting
osteochondroma is k/a peripheral
chondrosarcoma
Age- 25- 45 yrs( earlier than primary
chondrosarcoma)
Site- pelvis & shoulder girdle mc affected
C/F
Change in clinical symptom of a patient with
precursor lesion
Sudden onset of pain
Increase in size of swelling
Radiology
Osteochondroma- irregular mineralization,
increased thickness of cartilage cap( size of
cap > 2 cm in skeletally mature patient on
CT/MRI)
Enchondroma- destructive permeation of bone
and development of soft tissue mass
Periosteal chondrosarcoma
a/k/a juxtacortical chondrosarcoma
Arise from outer surface of bone possibly of
periostel origin
Periosteal chondrosarcoma
Dedifferentiated chondrosarcoma
High grade sarcoma(osteosarcoma,
fibrosarcoma, malignant fibrous
histiocytoma) adjacent to a low grade
chondrosarcoma
Radiological- aggressive radiolucent area
juxtaposed to typical chondrosarcoma
Low grade High grade spindle cells
chondrosarcma
Clear cell chondrosarcoma
Round cells with abundant clear cytoplasm
and distinct cytoplasmic border with
abundant background of cartilaginous matrix
Multinucleate giant cells are apparent
Strong tendency to arise in epiphysis
May have benign radiological feature and
confused with chondroblastoma or GCT
Epiphyseal Gaint Cell Tumor Chondroblastoma Clear cell
chondrosarcoma
Mesenchymal chondrosarcoma
High grade
Small round blue cells with islands of benign
appearing cartilage
Hemangiopericytomatous growth with
staghorn like vessels
Aggressive radiographic findings
Mesenchymal chondrosarcoma
Treatment
Summary
Chondrosarcomas represent a diverse spectrum
of diseases. They are tumors that should be
treated with definitive and complete surgical
resection if local control is to be achieved.
Although grade 1 lesions in the extremity can
be managed with curettage, incomplete
surgical resection of intermediate and high-
grade lesions puts the patient at risk for local
failure, metastases, and eventual death.
Low grade chondrosarcoma
controversial treatment
Extended curettage with use of
intraoperative adjuvant- only for low grade
chondrosarcoma confined to medullary
canal.
With soft tissue extention- treated similar to
high grade
High grade chondrosarcoma
Wide or radical resection or amputation
Primary wide resection without a biopsy(to
avoid chance of tumour contamination) is
indicated for any lesion in pelvis or
expendable location.
Local recurrence rate after tumour
contamination is high
After wide resection recurrence is < 10%
Chemotherapy
No role
Currently evaluated for treatment of
mesenchymal and dediffrentiated
chondrosarcoma
Radiotherapy
Limited role
For palliative purpose
Proton beam therapy(recent advance)
Used for skull base chondrosarcoma
Metastasis
Most local recurrence & pulmonary
metastasis occour within 1st 5 years of initial
presentation
Compared to other sarcomas
chondrosarcoma has high prevalance of local
recurrence or metastasis after 5 years hence
survival of patient be determined at 10 years
rather than 5 years
Pulmonary metastasis- wide surgical
resection
Prognosis mostly depends on size, grade &
location of tumour
More size poor prognosis
Higher grade- poor prognosis
Pelvic lesion- poor prognosis
Low grade chondrosarcoma- 10 year survival
rate> 90%
High grade chondrosarcoma- 10 year survival
rate 20-40%
Recurrence generally have higher grade than
primary malignancy bcoz slow growing
tumour and recurrence or mets cant be
detected years after primary procedure
Long term follow up with regular imaging of
operative site and chest
Work up for a case of chondrosarcoma
Diagnosis often on clinical & radiological
findings
Biopsy- controversial role
Laboraty investigations- nonspecific
Staging- CT chest
Treatment based on combined clinical,
radiological , histological grade findings
Biopsy
Performed only to confirm what in clinically
and radiologically obvious
Decision taken by an experienced
orthopaedic oncologist
Closed or open
Closed- taken from area of uncalcified matrix
disadvantage- chondrosarcoma highly
heterogeneous( high grade can have areas
of low grade)
Open biopsy sometimes advocated to
minimise potential sampling error
Pelvic lesion biopsy is generally not
recommended
Extended curettage
Simple curettage+ use of adjuvants
Adjuvants-phenol, nitrogen,polymethyl
methacrylate,argon beam coagulator
Cortical window-larger than lesion
Tumour scooped out
Cavity enlarged in each direction with burr hole
Copious irrigation of cavity
Use of adjuvant
Filling cavity-autogenous bone graft, allograft,
artificial bone graft substitute,demineralized
bone matrix, bone cement
Wide/negative margins are the goal of surgical resection
Limb salvage vs amputation
Every patient with tumor of the extremity should
be considered for limb salvage if the tumor can
be removed with an adequate margin and the
resulting limb is worth saving.

No justification for limiting the limb salvage


process based only on the prognosis
contraindication
Neurovascular involvement
Large size tumour
Displaced pathologic fracture(relative
contraindication)
Fungating and infected tumors
Recurrence of malignant tumors
Skeletal immaturity
Pulmonary metastasis is not a contraindication
of surgery
Contraindications of limb salvage are the
indications for amputation
RESECTION & RECONSTRUCTION
Resection-Wide margin
Reconstruction
Arthrodesis
Mobile joint reconstruction
Osteoatricular allograft reconstruction
Endoprosthetic reconstruction
Allograft prosthesis composite
reconstruction
Rotationplasty
Autoclaved bone tumour
Arthrodesis
Bone allografts, Vascularised autografts or both.
Provides a stable, durable reconstruction which
requires limited postoperative follow-up
Inherent disadvantages:
- Loss of joint function
- Increased energy expenditure
- Additional abnormal mechanical stress to other
joints
Osteoarticular allografts
Adv:
Ability to replace ligaments, tendons &
intraarticular structures.
As a temporary measure to preserve adjacent
physis till skeletal maturity e.g. Prox tibia
Disadv:
nonunion at graft host jxn.
fatigue #, articular collapse, dislocation,
degenerative jt. dis. & failure of ligament &
tendon attachments.
Endoprosthetic Reconstruction
Adv:
Predictable immediate stability
Quicker rehab
Increased durability better implants.
Incremental limb lengthening

Disadv:
Long term compl. if pt. is cured of disease.
polyetheylene wear inserts replaced.
Fatigue # at base of stem difficult to
remove
Allograft prosthesis composite reconstruction
Autoclaved bone tumour
Amputation
Specific technical difficulty for resection as well
as reconstruction of pelvic chondrosarcoma
Interilio-abdominal disarticulations
Conservative surgeries
Pelvic resection
I- iliac
II-periacetabular
III-obturator
IV-sacral
Eg- acetabulum+
iliac wing ka I,II
pelvic resection
H- added as suffix
if femoral head is
resected
Type I- no recontruction required ,limb
length discrepancy(1-2 cm) adjusted
with shoe raise
Type II- reconstruction with iliofemoral,
ischiofemoral arthrodesis or prosthesis
Type III- no reconstruction required
(pubis does not bear weight)
Type II resection reconstructed with
saddle prosthesis
Thank you

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