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8/3/2019 Chon Dro Sarcoma
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CHONDROSARCOMA
VIVEK PANDEY
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• Malignant tumor of cartilage producingcells.
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WHO CLASSIFICATION OF MALIGNANTCARTILAGENOUS TUMOURS
• Bone:
Chondrosarcoma;
central vs peripheral
primary vs secondary Juxtacortical( periosteal) Chondrosarcoma Dedifferentiated Chondrosarcoma Mesenchymal Chondrosarcoma
clear cell chondrosarcoma• Soft tissue:
Extraskeletal Myxoid
Mesenchymal
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Primary Chondrosarcoma
• Malignant cartilage tumor arising centrally in previouslynormal bone.
• Also k/a central or conventional chondrosarcoma.• 3rd MC primary malignancy of bone after myeloma &
osteosarcoma.
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• Age: tumor of adulthood
beyond 3rd decade
Peak incidence: 5th to 7th decade
M:F 2:1
Sites:Ilium followed by Proximal femur, proximal humerus,distal femur & ribs.
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Clinical features
• Pain : MC & often only presentation• > 50% have rest or night pain• Pathological fractures are rare
seen in 3 to 5 %
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Radiologic Findings
• Expansion of medullary portion of the bone& thickening of cortex,
• Endosteal scalloping with annular, punctate or commashaped stippled calcification.
• Located in metaphysis of femur, tibia & humerus• Cortical expansion & thickening are adaptive changes,
cortical disruption & soft tissue masses are aggressivechanges.
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CT Scan
• Low grade features• Dense calcification forming rings or spicules• Widespread or uniformly distributed calcifications• Eccentric lobular growth of a soft tissue mass.
• High grade features:• Faint amorphous calcification• Large non calcified areas• Concentric growth of a soft tissue mass
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Bone scan
• Murphy’s grading:• Grade 1: uptake < that in ASIS• Grade 2: uptake similar to that in ASIS• Grade 3: uptake > that in ASIS
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CT/MRI
• Endosteal scalloping & bone distruption – CT• MRI- intramedullary extent of tumor, soft tissue extension
& marrow replacement by the tumor
• BIPOSY• to determine the grade of disease• taken from ares of bone destruction & areas showing high
degree of endosteal scalloping & lysis
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Clinicopathologic Grading
• grade 1:- may be identical to benign enchondroma;- where as a low grade malignant appearing
cartilagenous tumor of extremities may actually be an
enchondroma, a centrally occurring malignant appearingenchondroma in an adult is more likely
represents a chondrosarcoma;- grade 2:- increased cellularity but rarely have mitotic
figures and are more locally aggressive, with a 15% to
20% rate of metastasis;- grade 3: (dedifferentiated and mesenchymal
types) mitotic figures with increased cellularity, leading toa 70% rate of metastasis;
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TREATMENT
• Chondrosarcoma in situ: intralesional excision• Chondrosarcoma with adaptive or aggressive changes:
wide resection is recommended for Grade 2 & grade 3 of long bones.
Chondrosarcoma of axial skeleton: wide excision
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Prognosis
• Histological grading
metastases seen in 10 % of grade 2 & 71 % of grade 3chondrosarcoma.
Pts with Grade 1 lesion completely resected: almost cured
Survival rates of 10 years were 89 % for Gr 1, 53 % for Gr 2& 38% for Gr 3.
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SECONDARY CHONDROSARCOMA
• Chondrosarcoma arising in known being precursor lesion .
• Precursor lesion: osteochondroma or enchondroma
• Pts with Olliers disease & Maffucci’s syndrome 25-30 %risk of developing chondrosarcoma.
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Clinical features
• pain• Swelling• Paresthesia• Pathological fracture
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Imaging
• Poorly defined, lytic , intraosseouslesion with associaatedcorticaal perforation & extraosseous extension into softtissue producing large mass.
• Cartilagenous portion is sharply distinct from permeative
& destructive component.
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HISTOPATHOLOGY
• Gross: lobulated low grade cartilagenous component isblue grey , central in location,
• Haemorrhagic high grade is extraosseous.
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Prognosis
• Very poor prognosis• 90% patients die with distant metastasis in 2 yrs
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MESENCHYMAL CHONDROSARCOMA
• Malignant tumor characteried by bimorphic patterncomposed of highly undifferentiated small round cells &islands of well differentiated hyaline cartilage.
• < 1 % of malignant bone tum.• <3-10% of all pri. Chondrosarcoma.• AGE: all ages with peak in 2nd & 3rd decade.• SEX: M:F Equal• SITES: Craniofacial bones, ribs, ilium 7 vertebrae are
common• CLINICAL FEATURES: pain & swelling
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