Chon Dro Sarcoma

8/3/2019 Chon Dro Sarcoma

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CHONDROSARCOMA

VIVEK PANDEY



• Malignant tumor of cartilage producingcells.



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



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.



• 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.



Clinical features

• Pain : MC & often only presentation• > 50% have rest or night pain• Pathological fractures are rare

seen in 3 to 5 %



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.



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



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



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



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;

http://www.wheelessonline.com/ortho/enchondroma

http://www.wheelessonline.com/ortho/enchondroma



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



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.



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.



Clinical features

• pain• Swelling• Paresthesia• Pathological fracture



Imaging

• Poorly defined, lytic , intraosseouslesion with associaatedcorticaal perforation & extraosseous extension into softtissue producing large mass.

• Cartilagenous portion is sharply distinct from permeative

& destructive component.



HISTOPATHOLOGY

• Gross: lobulated low grade cartilagenous component isblue grey , central in location,

• Haemorrhagic high grade is extraosseous.



Prognosis

• Very poor prognosis• 90% patients die with distant metastasis in 2 yrs



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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Chon Dro Sarcoma