chondrosarcoma c/f diagnosis treatment

1. CHONDROSARCOMA
DR. KUMAR SUPRASHANT
DNB RESIDENT
HINDU RAO HOSPITAL AND
NDMC MEDICAL COLLEGE

2. CHONDROSARCOMA
OVERVIEW
1.Defination
2.Frequency
3.Types
Defination c/f
Frequency radiology
Epidemiology histology
Etiology
4.Treatment

3. CHONDROSARCOMA
DEFINATION
Malignant tumour of cartilage producing cells

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

13. RING AND ARC CALCIFICATION

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

18. CHONDROSARCOMA ENCHONDROMA

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

21. Intramedullary
extention & soft
tissue extention

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

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

35. Periosteal chondrosarcoma

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

37. Low grade
chondrosarcma
High grade spindle cells

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

41. Mesenchymal chondrosarcoma

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

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

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.

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

67. Allograft prosthesis composite reconstruction

68. Autoclaved bone tumour

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

74. Thank you