CHONDROSARCOMA

&
SYNOVIAL SARCOMA
Dr.V.V.Narasimha Rao
1st year PG
Dept. of Orthopaedics
INTRODUCTION:

• Malignant cartilage forming tumor that does not produce osteoid

• May arise from osteochondroma
• Third most common bone malignancy after myeloma and
osteosarcoma
• Most common subtype of chondrosarcoma
• Usually ages 30 - 60 years, 75% males
INCIDNCDE:

• Chondrosarcoma is a tumor of adulthood and older

age.
• The majority of patients are older than 50 years of
age at diagnosis, 
• M/C Location : Proximal Locations –
PELVIS
Proximal Femur
Proxima Humerus

• Rarely occur In hand

Pathophysiology

• Amplification of MYC and AP-1transcription factors plays a vital role

in the pathogenesis of chondrosarcoma analyzed by cytogenetics. 
Histopathology

• Macroscopic findings:
• Chondrosarcomas are large tumors, usually greater than 4 cm in size .
• They have a translucent lobular, blue-grey, or white cut surface
corresponding to the presence of hyaline cartilage.
• There may be areas containing myxoid or mucoid material and cystic
changes.
• Yellow-white chalky areas of calcium deposit are commonly present
(mineralization). Erosion and destruction into soft tissue may be seen.
Microscopic findings:
• Abundant blue-grey cartilage matrix-production.
• Irregularly shaped lobules of cartilage varying in size and shape are
present.
• Fibrous bands separate these lobules or permeate bony trabeculae.
Calcified areas suggesting the presence of a pre-existing enchondroma
can often be found.
• The chondrocytes are atypical, with variable size and shape and contain
enlarged hyperchromatic nuclei with binucleation.
• Chondroid matrix liquefaction or myxoid changes are a common
feature of chondrosarcomas. 
primary chondrosarcoma
• low-grade, high-grade, dedifferentiated chondrosarcoma
• clear cell chondrosarcoma (see below)
• mesenchymal chondrosarcoma
secondary chondrosarcoma

•arises from benign cartilage lesions including       

•osteochondroma (<1% risk of malignant transfomation)
•multiple hereditary exostosis (1-10% risk of malignant
transformation)       
•enchondromas (1% risk of malignant transformation)
•Ollier's disease (25-40% risk of malignant transformation)
•Maffucci's (100% risk of malignant transformation)
• Chondrosarcomas are divided into three grades based upon their
histopathology:
• Grade
• Grade
• 85% of chondrosarcomas are grade 1 or 2
• 15% of chondrosarcomas are grade 3 or dedifferentiated chondrosarcoma 
• de-differentiated chondrosarcomas are high grade lesions which develop from low grade
chondroid lesions
• Chondrosarcoma sub-types:
1) Clear cell chondrosarcoma
2) Mesenchymal chondrosarcoma
• Radiographic Appearance:

Diagnostic

Araises from Medullary Cavity with irregular Matrix

Calcification

Punctate
Popcorn Pattern of calcification

Comma Shaped
More Aggressive with bone Destruction

Cortical Erosions

Periosteal reaction

oft Tissue Mass

• CT –
Endosteal Erosions & evidence of destructive lesion

Differentiate b/w Benign & malignant lesions

• SITE -
Size of Cartilaginous Cap of an Osteochondroma - >2
cms
• Differential Diagnosis
• Chondromyxoid fibroma
• Enchondroma
• Chondroblastic osteosarcoma
• Fracture callus
• HITSOLOGICALLY:

Malignant cells with abundant cartilaginous matrix

• BIOPSY:

Differentiate between from Enchondroma and Chondrosarcoma

• Malignant Diagnosis:

Hypercellularity

Plump nuclei

Bi nucleated cells

Permeative Pattern

Entrapment of bony trabeculae

• Histological Sub Types:

De Differentiated ChondroSarcoma

Clear cell Chondrosarcoma

Mesenchymal Chondrosarcoma

• <20% of all chondrosarcomas

• De – Differentiated ChondroSarcoma:

High Grade Sarcoma

Shows More aggressive Radiolucent area juxtaposed

on an otherwise typical chondrosarcoma

5yr survival rate - < 15% ( 2yrs)

• Clear cell Chondrosarcoma:

Low grade Malignancy

Round cells with abundant Clear Cytoplasm &

Distinct Cytoplasmic Borders with Background
with a background of cartiliginous matrix

Strong Tendency to araise from Epiphysis

• MESENCHYMAL CHONDROSARCOMA:

High Grade Tumor

Small , Round Blue cells with islands of benign appearing cartilage

Cellar Portions – “HemangioPericytomatous”

Staghorn vessels
TREATMENT:
• Low Grade :

 ?EXTENDED CURETTAGE

Only for Low Grade Lesions confined to medullary Canal

• HIGH GRADE:

 With Soft Tissue Extension

 Wide / Radical Resection / Amputation

 Avascular Cartilage - cells survive

Local Recurrence
• Lesions on Expendable Location:

Primary wide resection without a biopsy

( decreases Intra Operative Contamination)

 Local Recurrence – 10 %

Repeat Wide resection / wide amputation

• No Role of Chemotherapy

• Being used

• Long term follow up with regular imaging of operating site & chest is
imperative
• Prognosis Depends upon:

Size High Grade – 20 to 40%

Grade
Low Grade – 90%
Location
SYNOVIAL SARCOMA
• SITE:
Around ankle & knee

 Shoulder and hip joint

 Retropharyngeal area , Ant.abdominal wall , blood vessels , nerves

• 30 yrs of age

• Male : Female – 1 : 2

• Exact Etiology – unknown

• Activation of Proto Oncogene of inhibition of tumor suppressor genes

• Stains for epithelial membrane antigen and cytokeratin.

• Histologically:

biphasic: 20-30%
monophasic fibrous: 50-60%
monophasic epithelial: very rare
poorly differentiated: 15-25%
• MICROSCOPICALLY :

 Biphasic tumor ( Epithelial & Sarcomatous)

 Epithelial areas : Gland like spaces lined by cuboidal

or columnar cells.

 Sarcomatous Component : Spindle cells with a fibro

blast appearance

 Hyper Cellularity
• SYMPTOMS:

Swelling or mass accompanied by pain

 Limping & difficulty using legs , arms , hands, feet

• GROSSLY:

Well circumscribed , firm & greyish pink , focal calcification

Varigated appearance ,often with necrosis and hemorraghe

• Monophasic Synovial Sarcoma – one of the two
components .

• A poorly differntiated sarcoma : Greater degree of

cellularity, atypia & mitotic activity .

• Synovial sarcoma associated with chromosomal

translocation t(x;18) (p11.2;q11.2)
Large number of Mast cells

 Hyalinization

Calcification

 osseous metaplasia
• Local Recurrance

• Metastasize distantly ( lung & lymph nodes)

• Nodal metastases - 10 – 15 %
• Plain Radiograph:
 normal unless, large mass or contains dystrophic calcifications

• USG:
 non specific , with a heterogenous predominantly hypoechoic mass
• CT :
non-specific, similar to ultrasound.
appears as a soft tissue mass of heterogeneous density and
enhancement.
 CT is, of course, more sensitive to calcifications than either
radiographs or MRI.
• MRI :
 Modality of choice
PROGNOSIS:
• Heavy calcification

• Age ( better in young pts )

• Site ( distal lesions )

• Size ( < 5cms )

• Status of surgical margins

• Mitotic activity ( < 15 mitoses )

• Rhabdoid cells ( worse)

TREATMENT :
• Surgery :

 Complete Surgical Excision near by muscles and lymph nodes

- best

• Radiation :

 often used in conjunction with surgery

Radiation alone

Pre operatively
• Chemotherapy :

 Highly Effective

Ifosfamide and Doxorubcin

Thank you