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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-à-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
chondrosarcma
High grade spindle cells
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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Chondrosarcoma

  • 1. CHONDROSARCOMA DR. KUMAR SUPRASHANT DNB RESIDENT HINDU RAO HOSPITAL AND NDMC MEDICAL COLLEGE
  • 4. 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
  • 5. CHONDROSARCOMA TYPES • Primary chondrosarcoma • Secondary chondrosarcoma • Periosteal chondrosarcoma • Dedifferentiated chonrosarcoma • Clear cell chondrosarcoma • Mesnchymal chondrosarcoma
  • 6. 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
  • 7. • 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)
  • 8. Clinical feature • Pain • Palpable mass • Asymptomatic incidental finding • Pathological fracture( in high grade chondrosarcoma)
  • 9. 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
  • 10. Chondrosarcoma from iliac bone with soft tissue extention
  • 11. Axial CT image showing mass arising from iliac bone with chondroid calcification which suggest chondrosarcoma
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  • 13. RING AND ARC CALCIFICATION
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  • 15. 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
  • 16. 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
  • 17. Site- aggressive lesion in hand still considered benign whereas same amount of aggressive behaviour in pelvis or proximal femur is dignostic of chondrosarcoma
  • 19. CT scan • Differentiate benign from malignant lesion • For characterizatiion of lesion in anatomically complex areas- sacrum, pelvis
  • 20. 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
  • 22. 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
  • 23. PET scan • Standard uptake value (suv) may be helpful in diffrentiating benign from malignant catrilaginous lesion
  • 24. 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-à-vis enchondroma) Grade 2 - ↑ cellularity and nuclear atypia Grade 3- ↑ ↑ cellularity and pleomorphic anaplastic cells
  • 25. 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
  • 26. Intramedullary ,soft tiisue extention of tumour with calcification
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  • 30. 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
  • 31. • 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
  • 32. 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
  • 33. 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
  • 34. Periosteal chondrosarcoma a/k/a juxtacortical chondrosarcoma Arise from outer surface of bone possibly of periostel origin
  • 36. Dedifferentiated chondrosarcoma High grade sarcoma(osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma) adjacent to a low grade chondrosarcoma Radiological- aggressive radiolucent area juxtaposed to typical chondrosarcoma
  • 38. 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
  • 39. Epiphyseal Gaint Cell Tumor Chondroblastoma Clear cell chondrosarcoma
  • 40. Mesenchymal chondrosarcoma • High grade • Small round blue cells with islands of benign appearing cartilage • Hemangiopericytomatous growth with staghorn like vessels • Aggressive radiographic findings
  • 42. 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.
  • 43. 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
  • 44. 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%
  • 45. 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
  • 46. 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
  • 47. • Prognosis mostly depends on size, grade & location of tumour • More size – poor prognosis • Higher grade- poor prognosis • Pelvic lesion- poor prognosis
  • 48. • 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
  • 49. 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
  • 50. 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)
  • 51. Open biopsy – sometimes advocated to minimise potential sampling error Pelvic lesion – biopsy is generally not recommended
  • 52. 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
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  • 54. Wide/negative margins are the goal of surgical resection
  • 55. 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
  • 56. 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
  • 57. RESECTION & RECONSTRUCTION Resection-Wide margin Reconstruction Arthrodesis Mobile joint reconstruction • Osteoatricular allograft reconstruction • Endoprosthetic reconstruction • Allograft prosthesis composite reconstruction • Rotationplasty • Autoclaved bone tumour
  • 58. 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
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  • 63. 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.
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  • 65. 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
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  • 69. Amputation Specific technical difficulty for resection as well as reconstruction of pelvic chondrosarcoma • Interilio-abdominal disarticulations • Conservative surgeries
  • 70. Pelvic resection • I- iliac • II-periacetabular • III-obturator • IV-sacral
  • 71. • Eg- acetabulum+ iliac wing ka I,II pelvic resection • H- added as suffix if femoral head is resected
  • 72. • 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)
  • 73. Type II resection reconstructed with saddle prosthesis