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Chondrosarcoma

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Chondrosarcoma

Malignant cartilage-forming tumor - second most common primary bone malignancy with varied biological behavior

complete
Updated: 2025-12-24
High Yield Overview

CHONDROSARCOMA

Malignant Cartilage Tumor | Second Most Common Primary Bone Malignancy | Variable Biological Behavior

20%of all primary bone malignancies
50-70 yrspeak age incidence
Grade I: 90%10-year survival
Grade III: 29%10-year survival

Histological Grading

Grade I
PatternLow-grade, well-differentiated
TreatmentWide resection, excellent prognosis
Grade II
PatternIntermediate cellularity and atypia
TreatmentWide resection, good prognosis
Grade III
PatternHigh-grade, poorly differentiated
TreatmentWide resection, poor prognosis

Critical Must-Knows

  • Resistant to chemotherapy and radiotherapy - surgery is the only curative treatment
  • Histological grade is the most important prognostic factor determining survival
  • Distinguishing low-grade chondrosarcoma from enchondroma is challenging clinically and histologically
  • Pelvis and proximal femur are most common sites - central (medullary) subtype predominates
  • Pain in a previously asymptomatic cartilage lesion suggests malignant transformation

Examiner's Pearls

  • "
    Chondrosarcoma is radio-resistant and chemo-resistant - wide surgical resection is mandatory
  • "
    Pathological fracture is rare but indicates aggressive biology
  • "
    Dedifferentiated chondrosarcoma has biphasic pattern: low-grade cartilage plus high-grade sarcoma
  • "
    Pelvic chondrosarcomas have worse prognosis due to late presentation and difficulty achieving wide margins

Clinical Imaging

Imaging Gallery

Osteochondroma and Nora's lesion tumour characteristics. An osteochondroma is displayed in the X-ray (A). Microscopic analysis showed typical hyaline cartilage cup and growth plate-like bone prolifera
Click to expand
Osteochondroma and Nora's lesion tumour characteristics. An osteochondroma is displayed in the X-ray (A). Microscopic analysis showed typical hyaline Credit: Simon MJ et al. via BMC Musculoskelet Disord via Open-i (NIH) (Open Access (CC BY))
Radiologic and histologic examination of original tumors.A: L835 conventional radiography demonstrates a mildly expansile mixed lytic and sclerotic lesion in the distal radius with spiculated borders
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Radiologic and histologic examination of original tumors.A: L835 conventional radiography demonstrates a mildly expansile mixed lytic and sclerotic leCredit: van Oosterwijk JG et al. via BMC Cancer via Open-i (NIH) (Open Access (CC BY))
(A) Chest X-ray of a 37-year-old female who presented with a mass and pain in the anterior chest wall, however, had not exhibited any indication of a sternum mass prior to surgery. (B) Chest X-ray one
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(A) Chest X-ray of a 37-year-old female who presented with a mass and pain in the anterior chest wall, however, had not exhibited any indication of a Credit: He B et al. via Oncol Lett via Open-i (NIH) (Open Access (CC BY))
Dedifferentiated chondrosarcoma of the left distal femur in a 73-year-old man (Case 4).(A,B). Anteroposterior and lateral radiographs demonstrated chondral matrix mineralization, showing a low-grade c
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Dedifferentiated chondrosarcoma of the left distal femur in a 73-year-old man (Case 4).(A,B). Anteroposterior and lateral radiographs demonstrated choCredit: Open-i / NIH via Open-i (NIH) (Open Access (CC BY))
Clear-cell chondrosarcoma of proximal humerus - multimodality imaging
Click to expand
Clear-cell chondrosarcoma of proximal humerus demonstrating multimodality imaging. (A) AP shoulder X-ray showing diffuse sclerosis and periarticular changes. (B) Axial CT showing characteristic chondroid matrix mineralization with 'rings and arcs' pattern. (C-D) Axial MRI showing heterogeneous mass with areas of high T2 signal typical of cartilaginous tumors. (E-F) Coronal MRI demonstrating full tumor extent for surgical planning. Clear-cell chondrosarcoma is a rare low-grade subtype affecting the epiphysis.Credit: Elojeimy S et al., Radiol Case Rep - CC-BY

Critical Chondrosarcoma Exam Points

Treatment Resistance

Surgical resection is the ONLY curative treatment. Chondrosarcoma is notoriously resistant to both chemotherapy and radiotherapy. Wide margins are essential.

Grading is Prognostic

Histological grade determines survival. Grade I has 90% 10-year survival, Grade III only 29%. Grading is challenging - requires adequate tissue and expert pathology.

Enchondroma vs Chondrosarcoma

Distinction is notoriously difficult. Pain, size greater than 5cm, endosteal scalloping greater than two-thirds cortical thickness, and uptake on bone scan suggest malignancy.

Dedifferentiated Subtype

Worst prognosis subtype. Biphasic tumor with low-grade cartilage component plus high-grade non-cartilaginous sarcoma. Treated as high-grade sarcoma with neoadjuvant chemotherapy.

At a Glance

Chondrosarcoma is the second most common primary bone malignancy (20%), characterized by malignant cartilage formation. It is resistant to chemotherapy and radiotherapy - wide surgical resection is the ONLY curative treatment. Histological grading (I-III) is the most important prognostic factor: Grade I has 90% 10-year survival while Grade III drops to 29%. The pelvis and proximal femur are most common sites. Key diagnostic challenge: distinguishing low-grade chondrosarcoma from benign enchondroma - pain, size greater than 5cm, and endosteal scalloping greater than two-thirds cortical thickness suggest malignancy. Dedifferentiated chondrosarcoma (biphasic: low-grade cartilage plus high-grade sarcoma) has worst prognosis.

Quick Clinical Decision Guide

PresentationGradeSurgeryPrognosis
Asymptomatic lesion, incidental findingGrade I (well-differentiated)Wide resection with margins90% 10-year survival, excellent
Mild pain, enlarging massGrade II (intermediate)Wide resection, consider adjuvant if margin close81% 10-year survival, good
Severe pain, rapid growth, soft tissue massGrade III (poorly differentiated)Wide resection, consider chemotherapy29% 10-year survival, poor
Aggressive symptoms, pathological fractureDedifferentiated (biphasic)Wide resection plus neoadjuvant chemotherapy7-24% 5-year survival, very poor
Mnemonic

PUFFChondrosarcoma Anatomical Distribution

P
Pelvis
Most common site - 30-40% of cases
U
Upper femur
Proximal femur and femoral shaft
F
Flat bones
Scapula, ribs (especially costal cartilage)
F
Far from hands/feet
Rare in distal extremities

Memory Hook:Chondrosarcoma goes for the PUFF - Pelvis and pUoximal femur are favorite sites, unlike enchondroma which prefers hands!

Mnemonic

PESTFeatures Suggesting Malignancy (Enchondroma vs Chondrosarcoma)

P
Pain
New or progressive pain in cartilage lesion
E
Endosteal scalloping
Greater than two-thirds cortical thickness
S
Size
Lesion greater than 5cm diameter
T
Thallium/Technetium uptake
Positive uptake on bone scan or PET

Memory Hook:When an enchondroma becomes a PEST (painful, eroding, sizable, and tracer-positive), suspect chondrosarcoma!

Mnemonic

CCPM-DChondrosarcoma Subtypes

C
Central (Conventional)
Most common - 85%, medullary origin
C
Clear cell
Benign-appearing, epiphyseal, young patients
P
Periosteal
Surface lesion, better prognosis
M
Mesenchymal
Young patients, HEY1-NCOA2 fusion
D
Dedifferentiated
Worst prognosis - biphasic tumor

Memory Hook:CCPM-D covers Central (common), Clear cell (epiphysis), Periosteal (surface), Mesenchymal (young), and Dedifferentiated (deadly)!

Overview and Epidemiology

Chondrosarcoma is a malignant tumor characterized by the production of cartilaginous matrix by neoplastic cells. It is the second most common primary malignant bone tumor after osteosarcoma, accounting for approximately 20% of all primary bone malignancies. The annual incidence is approximately 1 per million population.

Why Chondrosarcoma Matters

Chondrosarcoma represents a unique challenge in orthopaedic oncology because it is resistant to both chemotherapy and radiotherapy, making surgical resection the only curative option. The difficulty in distinguishing low-grade chondrosarcoma from benign enchondroma creates diagnostic and treatment dilemmas. Histological grading is the most important prognostic factor.

Demographics

  • Peak age: 50-70 years (conventional type)
  • Gender: Male predominance (2:1 ratio)
  • Clear cell and mesenchymal: Younger patients (20-40 years)
  • Rare in children: Under 1% of cases

Anatomical Distribution

  • Pelvis: 30-40% (most common site)
  • Proximal femur: 20-30%
  • Ribs and scapula: 10-15%
  • Proximal humerus: 10%
  • Rare: Hands and feet (unlike enchondroma)

Pathophysiology and Mechanisms

Cartilage Tumor Biology

Chondrosarcoma arises from malignant transformation of chondrocytes. Understanding cartilage biology explains the treatment resistance of these tumors.

Cartilage Matrix

Avascular tissue - primary reason for chemotherapy and radiotherapy resistance:

  • No blood vessels penetrate cartilage matrix
  • Poor drug delivery to tumor cells
  • Hypoxic environment reduces radiotherapy efficacy
  • Drug resistance proteins highly expressed

Tumor Cell Characteristics

  • Low mitotic rate: Especially Grade I lesions
  • Abundant matrix production: Proteoglycans, collagen type II
  • Low metabolic activity: Contributes to imaging patterns
  • Lobulated architecture: Tumor nodules separated by fibrous septa

Why Surgery is the Only Option

The avascular nature of cartilage matrix creates a physical barrier to chemotherapy drug delivery. Even high-grade dedifferentiated chondrosarcomas receive chemotherapy for the NON-cartilaginous high-grade component, not the cartilage component itself.

Common Anatomic Sites and Biological Behavior

Anatomic RegionFrequencyTypical GradeChallenges
Pelvis (ilium, acetabulum)30-40%Grade II-IIILate presentation, difficult margins, higher recurrence
Proximal femur20-30%Grade I-IIReconstruction challenges, risk to neurovascular bundle
Ribs and sternum10-15%Grade I-IIChest wall resection, respiratory compromise
Proximal humerus10%Grade I-IIBrachial plexus proximity, rotator cuff sacrifice

Classification Systems

Histological Grading System

Histological grading is the most important prognostic factor for chondrosarcoma, directly correlating with survival, metastatic potential, and local recurrence risk.

Histopathology of chondrosarcoma showing cartilaginous lobules
Click to expand
Histopathology of chondrosarcoma: Multiple lobules of cartilaginous tumor cells within lacunae surrounded by blue-purple chondroid matrix. This low-grade chondrosarcoma shows relatively uniform chondrocytes with mild atypia. Grading based on cellularity, nuclear atypia, and mitotic activity determines prognosis and treatment approach.Credit: Open-i (NIH) - CC BY 4.0
GradeCellularityNuclear FeaturesMitoses10-Year Survival
Grade I (Low)Mildly increasedMinimal atypia, small nucleiAbsent to rare90%
Grade II (Intermediate)Moderately increasedModerate atypia, enlarged nucleiOccasional81%
Grade III (High)Markedly increasedSevere atypia, pleomorphicFrequent29%

Grading Challenges

Adequate tissue sampling is critical. Needle biopsy may sample only low-grade areas in a heterogeneous tumor. Up to 10% of tumors show grade progression in different areas. Central core needle biopsy should target the most aggressive-appearing area on imaging (highest T2 signal, cortical destruction, soft tissue mass).

Chondrosarcoma Subtypes

Central (Conventional) Chondrosarcoma

Most common subtype (85%) arising from medullary cavity.

Characteristics

  • Arises de novo (75%) or from enchondroma (25%)
  • Peak age: 50-70 years
  • Pelvis and proximal long bones
  • Lobulated hyaline cartilage matrix

Imaging Features

  • Central medullary lesion
  • Rings and arcs calcification pattern
  • Endosteal scalloping (greater than two-thirds suggests malignancy)
  • Soft tissue mass in higher grades

Primary vs Secondary:

  • Primary: Arises de novo in previously normal bone (75%)
  • Secondary: Malignant transformation of enchondroma (25%) or osteochondroma (rare)

Secondary chondrosarcomas should be suspected when a long-standing asymptomatic cartilage lesion becomes painful or enlarges.

Clear Cell Chondrosarcoma

Rare low-grade variant (under 2%) with distinctive features.

Unique Features

  • Epiphyseal location (unlike typical chondrosarcoma)
  • Young patients: 20-40 years
  • Cells with clear cytoplasm (glycogen)
  • Can mimic chondroblastoma

Clinical Behavior

  • Low-grade malignancy
  • Local recurrence: 15-20%
  • Metastases: Rare (under 5%)
  • Excellent prognosis with adequate resection

Differential diagnosis: Chondroblastoma (benign, younger age, painful, epiphyseal). Clear cell chondrosarcoma cells are larger with more abundant clear cytoplasm.

Periosteal (Juxtacortical) Chondrosarcoma

Surface lesion arising from periosteum.

Characteristics

  • Arises on bone surface (not medullary)
  • Hands, feet, and long bone metaphysis
  • Usually low-grade (Grade I-II)
  • Better prognosis than central type

Treatment

  • Wide local excision
  • Preserve medullary canal if possible
  • Low recurrence with adequate margins
  • Metastases very rare

Periosteal chondrosarcomas have a better prognosis than central chondrosarcomas of the same grade due to earlier detection and easier achievement of wide margins.

Mesenchymal Chondrosarcoma

Rare high-grade variant (under 5%) with unique characteristics.

Pathology

  • Biphasic: Small round blue cells plus cartilage
  • HEY1-NCOA2 fusion (diagnostic)
  • Young patients: 20-30 years
  • Ribs, jaw, pelvis common sites

Clinical Behavior

  • High-grade malignancy
  • Early metastases (lung, bone)
  • Local recurrence: 40-50%
  • 5-year survival: 55%

Treatment: Wide resection plus chemotherapy (unlike conventional chondrosarcoma, mesenchymal type may respond to chemotherapy used for Ewing sarcoma).

Dedifferentiated Chondrosarcoma

Worst prognosis subtype (5-10% of chondrosarcomas).

Pathology

  • Biphasic tumor: Low-grade cartilage plus high-grade non-cartilaginous sarcoma
  • High-grade component: Osteosarcoma, fibrosarcoma, or undifferentiated
  • Older patients: 60-70 years
  • Pelvis and femur most common

Prognosis

  • Extremely poor prognosis
  • 5-year survival: 7-24%
  • Early metastases (lung)
  • Local recurrence: 50%

Imaging Clue

Dedifferentiated chondrosarcoma should be suspected when imaging shows a biphasic pattern: typical chondroid lesion with adjacent non-mineralized aggressive soft tissue mass. The high-grade component shows lytic destruction and soft tissue extension.

Treatment: Wide resection plus neoadjuvant chemotherapy (treat as high-grade sarcoma despite cartilage component).

Clinical Presentation

Symptoms

  • Pain: Dull, aching (70-80% of patients)
  • Mass: Palpable swelling (50%)
  • Pathological fracture: Rare (5-10%) but indicates aggressive biology
  • Incidental finding: Asymptomatic lesion on imaging

Duration

  • Slow progression: Months to years typical
  • Pain in enchondroma: Suspect malignant transformation
  • Rapid onset symptoms: Higher-grade lesions
  • Pelvic lesions: Late presentation due to deep location

Red Flags for Malignancy

Distinguish enchondroma from chondrosarcoma:

  • New pain in a previously asymptomatic cartilage lesion
  • Progressive pain despite conservative treatment
  • Lesion size greater than 5cm
  • Axial skeleton or proximal long bone location (enchondromas favor hands)
  • Cortical destruction or soft tissue mass
  • Age over 40 years with new cartilage lesion

Physical Examination

Local Findings

  • Palpable mass: Firm, deep, fixed to bone
  • Tenderness: Over lesion site
  • Range of motion: May be restricted if juxta-articular
  • Pathological fracture: Deformity, crepitus (rare)

Systemic Assessment

  • General condition: Usually well (not systemically unwell)
  • Lymph nodes: Not typically involved
  • Chest examination: Metastases rare at presentation
  • Neurovascular status: Assess for compression

Investigations and Imaging

Proximal femur chondrosarcoma on plain radiograph
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AP hip radiograph showing proximal femur chondrosarcoma. Note the expansile lesion in the intertrochanteric region with cortical thinning and internal chondroid matrix mineralization (rings and arcs pattern). The proximal femur and pelvis are the most common sites for conventional chondrosarcoma. Wide surgical resection is the treatment of choice as chondrosarcoma is resistant to chemotherapy and radiotherapy.Credit: Min L et al., Onco Targets Ther - CC-BY
Calcaneal chondrosarcoma with X-ray and CT imaging
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Calcaneal chondrosarcoma demonstrating multimodality imaging. (A) Lateral calcaneus X-ray showing heterogeneous lytic lesion. (B-C) Sagittal and axial CT showing expansile lesion with intracystic calcifications and cortical breach - features that distinguish this from benign cartilage lesions. (D) Post-treatment lateral view. Chondrosarcoma of the foot is rare but follows the same surgical principles as other locations.Credit: Open-i / NIH - CC-BY

Diagnostic Imaging Protocol

First LinePlain Radiographs

Essential initial investigation. Two orthogonal views of entire bone.

Classic findings:

  • Matrix: Rings and arcs calcification (popcorn pattern)
  • Endosteal scalloping: Greater than two-thirds cortical thickness suggests malignancy
  • Cortical destruction: Higher grades
  • Soft tissue mass: Mineralized in higher grades
  • Pathological fracture: Rare but indicates aggressive biology

Location: Metaphyseal or diaphyseal (central type), epiphyseal (clear cell).

Second LineMRI Whole Bone

Gold standard for local staging. MRI of entire bone with joint above and below.

Key sequences:

  • T1: Low signal (cartilage)
  • T2/STIR: High signal (lobulated appearance)
  • T1 post-contrast: Septal and nodular enhancement

Critical features to assess:

  • Extent of medullary involvement
  • Cortical destruction and soft tissue extension
  • Neurovascular encasement
  • Joint invasion (contraindicates limb salvage)
  • Skip lesions (rare in chondrosarcoma)
StagingCT Chest and Whole-Body Imaging

CT chest: High-resolution for lung metastases (site of 90% of metastases).

Whole-body imaging:

  • PET-CT: Useful for detecting metastases and assessing grade (higher SUV in high-grade)
  • Bone scan: May show uptake (more common in malignant lesions vs enchondroma)
  • Skeletal survey: Alternative in pediatric patients
BiopsyTissue Diagnosis

Core needle biopsy preferred (performed by or in consultation with treating surgeon).

Critical biopsy principles:

  • Longitudinal approach in line with definitive incision
  • Avoid neurovascular structures
  • Sample most aggressive area (use imaging to guide - high T2 signal, cortical destruction)
  • Adequate tissue: Multiple cores from different areas if heterogeneous
  • Frozen section: Not reliable for grading chondrosarcoma

Biopsy Tract Contamination

The biopsy tract must be excised en bloc with definitive resection. Poorly planned biopsy can compromise limb salvage surgery. All biopsies should be performed at or in consultation with the sarcoma treatment center.

Imaging Features: Enchondroma vs Chondrosarcoma

Distinguishing Benign from Malignant Cartilage Lesions

FeatureEnchondroma (Benign)Chondrosarcoma (Malignant)
LocationHands and feet (60%), asymptomaticPelvis, proximal femur, ribs (axial skeleton)
SizeUsually under 3-4cmOften greater than 5cm
PainAbsent (unless pathological fracture)Present in 70-80% (dull, aching)
Endosteal scallopingLess than one-third cortical thicknessGreater than two-thirds cortical thickness
CortexIntact or mildly thinnedDestroyed or breached (higher grades)
Soft tissue massAbsentPresent in Grade II-III
Bone scanNo uptake or minimalUptake present (more intense in higher grades)
MRI T2 signalLobulated high signal, thin septaThick irregular septa, nodular soft tissue component

Staging

Enneking Surgical Staging System

Whole-body bone scan showing metastatic chondrosarcoma
Click to expand
Whole-body bone scan/PET showing metastatic chondrosarcoma: Multiple areas of increased uptake throughout the skeleton indicating widespread bony metastases. Although chondrosarcoma metastasizes less frequently than osteosarcoma, when it occurs it portends poor prognosis. Staging workup requires chest CT (pulmonary metastases) and bone scan.Credit: Open-i (NIH) - CC BY 4.0

Chondrosarcoma is staged using the Enneking staging system for musculoskeletal sarcomas.

StageGradeSiteMetastases5-Year Survival
IALow (G1)Intracompartmental (T1)None (M0)Greater than 90%
IBLow (G1)Extracompartmental (T2)None (M0)80-90%
IIAHigh (G2-G3)Intracompartmental (T1)None (M0)50-70%
IIBHigh (G2-G3)Extracompartmental (T2)None (M0)30-50%
IIIAnyAnyPresent (M1)Under 20%

Compartmental status:

  • Intracompartmental (T1): Confined within bone cortex or within a single anatomic compartment
  • Extracompartmental (T2): Extends beyond cortex into soft tissues or crosses major fascial planes

Management

📊 Management Algorithm
chondrosarcoma management algorithm
Click to expand
Management algorithm for chondrosarcomaCredit: OrthoVellum

Fundamental Principle

Surgery is the ONLY curative treatment for chondrosarcoma. Unlike osteosarcoma and Ewing sarcoma, conventional chondrosarcoma is resistant to both chemotherapy and radiotherapy. Wide surgical resection with negative margins is essential.

Treatment Algorithm by Grade and Location

Grade I Chondrosarcoma Management

Goals: Wide local excision with negative margins while preserving function.

Treatment Steps

Step 1Staging
  • MRI of entire bone
  • CT chest for staging
  • Consider PET-CT if diagnosis uncertain
Step 2Surgical Planning
  • Intralesional curettage is CONTRAINDICATED (high recurrence)
  • Wide resection with 1-2cm margin
  • Limb salvage usually achievable
  • Reconstruction: Allograft, endoprosthesis, or arthrodesis
Step 3Adjuvant Treatment
  • No chemotherapy (ineffective)
  • No radiotherapy unless unresectable with positive margins
  • Close surveillance for recurrence
Long-termFollow-up
  • Years 1-2: Every 3-4 months (chest X-ray, local imaging)
  • Years 3-5: Every 6 months
  • Beyond 5 years: Annually (late recurrences possible)

Grade I Treatment Pearl

Even Grade I (low-grade) chondrosarcoma requires wide resection, not curettage. Intralesional treatment has recurrence rates of 50-90%. Some surgeons advocate observation alone for small (under 3cm) asymptomatic Grade I lesions in expendable bones, but this is controversial.

Grade II-III Chondrosarcoma Management

Goals: Wide resection with negative margins, manage metastatic disease.

Treatment Steps

Step 1Staging
  • MRI entire bone
  • CT chest (essential - lung metastases in 10-20%)
  • PET-CT or bone scan for distant metastases
Step 2Surgical Resection
  • Wide resection with 2-3cm margins
  • En bloc resection of entire tumor with surrounding cuff of normal tissue
  • Biopsy tract must be excised
  • Neurovascular sacrifice may be required
Step 3Reconstruction
  • Endoprosthesis: Proximal femur, distal femur (immediate weight-bearing)
  • Allograft-prosthetic composite: Joint reconstruction with bone stock
  • Massive allograft: Preserve bone stock for young patients
  • Amputation: If neurovascular sacrifice required or failed limb salvage
Step 4Adjuvant Therapy
  • Chemotherapy: Generally ineffective, NOT routinely used
  • Radiotherapy: Only for unresectable tumors or positive margins (50-70 Gy)
  • Metastasectomy: Selected lung metastases may be resected

Margin Status is Critical

Margin status is the most important surgical factor for local control. Positive margins increase local recurrence risk from under 10% (negative margins) to 40-60% (positive margins). Re-resection should be considered if positive margins and anatomically feasible.

Dedifferentiated Chondrosarcoma Management

Treat as high-grade sarcoma despite cartilage component.

Treatment Steps

Step 1Staging
  • Full staging as per high-grade sarcoma protocol
  • CT chest, PET-CT
  • MRI entire bone and adjacent joint
Step 2Neoadjuvant Chemotherapy
  • Chemotherapy BEFORE surgery (unlike conventional chondrosarcoma)
  • Regimens: Doxorubicin/ifosfamide or high-dose methotrexate
  • Rationale: Treat high-grade non-cartilaginous component
  • Assess response with imaging after 2-3 cycles
Step 3Surgery
  • Wide resection with negative margins
  • Often requires amputation due to aggressive local spread
  • Limb salvage: Consider if good response to chemotherapy
Step 4Adjuvant Therapy
  • Complete chemotherapy protocol (total 6 cycles)
  • Radiotherapy for positive margins
  • Aggressive surveillance (high metastatic rate)

Dedifferentiated Subtype Treatment

Dedifferentiated chondrosarcoma is the ONLY chondrosarcoma subtype that receives chemotherapy. The high-grade non-cartilaginous component (osteosarcoma, fibrosarcoma, or undifferentiated) may respond to chemotherapy, unlike the cartilage component.

Pelvic Chondrosarcoma Management

Most challenging location - late presentation and difficult resection.

Treatment Steps

Step 1Assessment
  • CT with 3D reconstruction for surgical planning
  • MRI pelvis: Assess neurovascular involvement, sacral plexus
  • Angiography: Consider preoperative embolization
  • Assess resectability (Type I-IV pelvic resection)
Step 2Surgical Resection
  • Type I: Iliac wing resection (best prognosis)
  • Type II: Periacetabular resection (most common)
  • Type III: Pubic rami resection
  • Type IV: Sacral resection (worst prognosis)

Challenges:

  • Achieving adequate margins (often anatomically impossible)
  • Massive blood loss (type and crossmatch 6-10 units)
  • Sacral plexus sacrifice may be required
Step 3Reconstruction
  • Type I: No reconstruction required (non-weight-bearing)
  • Type II: Saddle prosthesis or custom implant
  • Hip transposition: Proximal femur to remaining ilium
  • No reconstruction: If limited life expectancy

Pelvic Chondrosarcoma Prognosis

Pelvic chondrosarcomas have worse prognosis than appendicular lesions (5-year survival 30-60% vs 70-90%) due to late presentation, difficulty achieving wide margins, and higher local recurrence (30-50%).

Surgical Technique Principles

Surgical Technique

Preoperative Planning

Imaging Assessment

  • MRI entire bone: Determine extent, soft tissue involvement
  • CT for bony anatomy: Assess cortical destruction, plan osteotomy levels
  • Angiography: Consider for pelvic lesions (preoperative embolization)
  • 3D reconstruction: Essential for complex pelvic resections

Surgical Planning

  • Margin planning: 1-2cm for Grade I, 2-3cm for higher grades
  • Biopsy tract excision: Plan en bloc excision of tract
  • Reconstruction strategy: Endoprosthesis, allograft, or arthrodesis
  • Blood products: Type and cross 4-6 units (more for pelvic resections)

Frozen Section Margins

Intraoperative frozen section is UNRELIABLE for assessing margins in chondrosarcoma. Cartilage matrix is difficult to section, and distinguishing normal from Grade I chondrosarcoma is challenging even on permanent sections. Plan adequate margins based on preoperative imaging.

Wide Resection Technique

Surgical Steps

Step 1Exposure

Approach: Extensile approach allowing proximal and distal control.

  • Identify and protect neurovascular structures
  • Proximal control before tumor manipulation
  • Avoid breaching tumor capsule or reactive zone
Step 2En Bloc Resection

Resection of tumor with cuff of normal tissue:

  • Osteotomies 1-2cm (Grade I) or 2-3cm (Grade II-III) from tumor on imaging
  • Preserve neurovascular bundle if not encased
  • Include biopsy tract in resection specimen
  • Soft tissue cuff around any cortical breach
Step 3Specimen Handling

Orient specimen for pathologist:

  • Mark margins with sutures or clips
  • Photograph specimen with orientation markers
  • Immediate formalin fixation (DO NOT freeze cartilage specimens)

Do's

  • Wide margins based on preoperative plan
  • Proximal control before tumor manipulation
  • Excise biopsy tract en bloc
  • Document margins with photos and marking

Don'ts

  • No intralesional curettage - even for Grade I
  • No frozen section reliance for margin assessment
  • No violation of reactive zone during dissection
  • No freezing of specimen (damages cartilage for histology)

Reconstruction After Resection

ReconstructionIndicationsAdvantagesDisadvantages
EndoprosthesisJuxta-articular lesions, older patientsImmediate stability, early mobilization, predictable outcomesLimited lifespan (10-15 years), infection risk, dislocation
Massive allograftYoung patients, preserve bone stockBiologic reconstruction, potential for remodelingFracture (15-20%), nonunion, infection, slow incorporation
Allograft-prosthetic compositeLarge metaphyseal defectsCombines stability of prosthesis with bone stock of allograftComplex surgery, higher complication rate
ArthrodesisExpendable joints (wrist, ankle)Durable, infection-resistant, good functionLoss of motion, adjacent joint arthritis

Complications

Surgical Complications

ComplicationIncidenceRisk FactorsManagement
Local recurrence10% (negative margins) to 40-60% (positive margins)Positive margins, high grade, inadequate resectionRe-resection if feasible, amputation for failed limb salvage
Surgical site infection5-15% (higher for pelvic resections)Massive reconstruction, pelvic surgery, diabetesAntibiotics, debridement, implant retention if possible
Neurovascular injury5-10%Pelvic location, tumor encasementIntraoperative recognition and repair, accept deficit if planned sacrifice
Prosthetic dislocation5-10% for endoprosthesisAbductor sacrifice, large head size, patient factorsClosed reduction, constrained liner, revision if recurrent
Allograft fracture15-20% at 10 yearsHigh-impact activities, slow incorporation, osteoporosisProtected weight-bearing initially, ORIF if fracture occurs
Pulmonary metastases10% (Grade I) to 70% (Grade III)High grade, positive margins, local recurrenceSurveillance CT chest, metastasectomy if resectable oligometastatic disease

Local Recurrence

Local recurrence is the most common cause of treatment failure. Most recurrences (70%) occur within first 3 years, but late recurrences beyond 5 years are possible. Inadequate surgical margins are the primary cause. Positive margin re-resection should be strongly considered.

Postoperative Care and Rehabilitation

Postoperative Rehabilitation After Endoprosthetic Reconstruction

Rehabilitation Timeline

PostoperativeImmediate (Day 0-3)

Goals: Pain control, prevent complications

  • DVT prophylaxis (LMWH for 6 weeks)
  • Wound drain management (remove when output under 30ml per 24 hours)
  • Mobilize day 1-2 with physiotherapy
  • Hip precautions if proximal femoral replacement (no flexion over 90 degrees)
Protected MobilizationEarly (Weeks 1-6)

Weight-bearing: Immediate weight-bearing as tolerated (endoprosthesis provides immediate stability)

  • Gait training with walking aids
  • ROM exercises within precautions
  • Wound healing (sutures removed 2-3 weeks)
  • Monitor for infection, dislocation
Progressive FunctionIntermediate (Weeks 6-12)

Goals: Restore functional independence

  • Wean walking aids as strength improves
  • Progressive resistance exercises
  • Return to ADLs (driving at 6-8 weeks if safe)
  • No high-impact activities (running, jumping - lifelong)
MaintenanceLong-term (Beyond 12 weeks)

Surveillance and monitoring:

  • Maintain muscle strength and joint ROM
  • Annual prosthesis surveillance (X-ray for loosening, wear)
  • Low-impact activities encouraged (swimming, cycling)
  • Lifelong activity modifications (avoid high-impact sports)

Postoperative Rehabilitation After Massive Allograft

Rehabilitation Timeline

PostoperativeImmediate (Day 0-3)

Same as endoprosthesis protocol for early mobilization and prophylaxis.

Allograft ProtectionProtected (Weeks 0-12)

Weight-bearing: Protected weight-bearing (50%) for 12 weeks (allograft is NOT immediately stable like endoprosthesis)

  • Walking aids mandatory
  • Avoid twisting, high stress
  • Monitor for fracture signs (pain, loss of function)
Incorporation PhaseProgressive (Weeks 12-26)

Weight-bearing: Progress to full weight-bearing by 6 months

  • Serial X-rays for union assessment
  • If delayed union, consider bone stimulation
  • Gradual increase in activities
MonitoringLong-term (Beyond 6 months)

Annual surveillance:

  • X-rays for fracture, nonunion, collapse
  • Allograft fracture risk 15-20% at 10 years
  • Activity restrictions less stringent than endoprosthesis once fully incorporated

Allograft vs Endoprosthesis Rehabilitation

Key difference: Endoprosthesis allows immediate full weight-bearing (metal implant provides instant stability), while allograft requires protected weight-bearing for 12 weeks (bone graft needs time to incorporate and remodel).

Surveillance Protocol

Years 1-2 (High Risk)

Every 3 months:

  • Clinical examination
  • Chest X-ray (PA and lateral)
  • Local imaging (X-ray or MRI)
  • 70% of recurrences occur within first 3 years

Years 3-5

Every 6 months:

  • Clinical examination
  • Chest X-ray
  • Local imaging
  • CT chest if concerning findings

Beyond 5 Years

Annually:

  • Clinical examination
  • Chest X-ray
  • Local imaging
  • Late recurrences possible - lifelong surveillance

Reconstruction Monitoring

Annual X-rays:

  • Endoprosthesis: Loosening, wear, subsidence
  • Allograft: Fracture, nonunion, collapse
  • Earlier imaging if pain or functional decline

Role of Radiotherapy

Limited Indications

Chondrosarcoma is radio-resistant. Radiotherapy is NOT standard treatment.

Consider radiotherapy only for:

  • Unresectable tumors (skull base, axial spine)
  • Positive margins when re-resection not feasible
  • Palliation of metastases

Specialized Techniques

High-dose radiotherapy required:

  • Proton beam therapy (skull base lesions)
  • Carbon ion radiotherapy (emerging)
  • Dose: 60-70 Gy conventional, 70-80 CGE proton

Conventional photon radiotherapy: Limited efficacy, local control under 50%

Prognosis and Outcomes

Prognostic Factors

FactorFavorable PrognosisUnfavorable Prognosis
Histological GradeGrade I (low-grade)Grade III or dedifferentiated
Anatomic SiteAppendicular skeleton (extremities)Axial skeleton (pelvis, spine, ribs)
Surgical MarginsWide negative margins (greater than 2cm)Intralesional or positive margins
Tumor SizeLess than 5cmGreater than 10cm
AgeUnder 40 yearsOver 60 years
Metastases at PresentationNone (M0)Present (M1) - lungs, bone
SubtypePeriosteal or clear cellDedifferentiated or mesenchymal

Most Important Prognostic Factor

Histological grade is the single most important prognostic factor for chondrosarcoma, more important than size or anatomic location. Grade I has 90% 10-year survival vs Grade III 29%. This emphasizes the critical importance of adequate biopsy tissue for accurate grading.

Metastatic Disease and Recurrence

Metastatic Pattern

  • Lungs: 90% of metastases
  • Bone: 10% (especially with higher grades)
  • Late metastases: Can occur 5-10 years after resection
  • Metastatic rate: 10% (Grade I), 30% (Grade II), 70% (Grade III)

Local Recurrence

  • Adequate margins: 10% recurrence
  • Marginal/positive margins: 40-60% recurrence
  • Time to recurrence: Average 2-3 years (range: 6 months to 10 years)
  • Management: Re-resection if feasible, amputation if limb salvage fails

Survival by Grade

Survival Outcomes

Grade5-Year Survival10-Year SurvivalMetastatic Rate
Grade I90-95%90%5-10%
Grade II70-85%81%25-30%
Grade III35-55%29%70-75%
Dedifferentiated10-25%Under 10%Over 90%

Evidence Base and Key Studies

Histologic Grading of Chondrosarcoma: A Prognostic Analysis

3
Evans HL, Ayala AG, Romsdahl MM • Cancer (1977)
Key Findings:
  • Landmark study establishing 3-tier grading system for chondrosarcoma
  • Grading based on cellularity, nuclear atypia, and mitotic activity
  • Clear correlation between grade and survival: Grade I 90%, Grade II 81%, Grade III 29% at 10 years
  • Grading is the most important prognostic factor for chondrosarcoma
Clinical Implication: Established histological grading as the foundation for chondrosarcoma prognosis and treatment planning.
Limitation: Retrospective study; grading can be subjective and requires expert musculoskeletal pathology.

Chondrosarcoma of Bone: A Long-Term Follow-Up Study of 165 Patients

3
Lee FY, Mankin HJ, Fondren G • Clinical Orthopaedics and Related Research (1999)
Key Findings:
  • 165 patients with chondrosarcoma, mean follow-up 10 years
  • Local recurrence: 27% overall (5% Grade I, 18% Grade II, 55% Grade III)
  • Metastases: 22% overall (strongly correlated with grade)
  • Margin status critical: Intralesional margins 47% recurrence vs wide margins 11% recurrence
Clinical Implication: Emphasized importance of adequate surgical margins - wide resection mandatory even for low-grade lesions.
Limitation: Single-institution study; heterogeneous treatment protocols over long time period.

Dedifferentiated Chondrosarcoma: Prognostic Factors and Outcome

3
Grimer RJ, Gosheger G, Taminiau A • Journal of Bone and Joint Surgery (American) (2007)
Key Findings:
  • Multicenter study of 337 dedifferentiated chondrosarcomas
  • 5-year survival: 24% (significantly worse than conventional chondrosarcoma)
  • Metastases present in 90% of deaths (primarily lungs)
  • Chemotherapy improved survival in some patients (from 7% to 18% at 5 years)
Clinical Implication: Dedifferentiated chondrosarcoma should be treated as high-grade sarcoma with neoadjuvant chemotherapy, not as conventional chondrosarcoma.
Limitation: Retrospective multicenter study; heterogeneous chemotherapy protocols.

Pelvic Chondrosarcoma: A Review of 64 Cases

3
Fiorenza F, Abudu A, Grimer RJ • Journal of Bone and Joint Surgery (British) (2002)
Key Findings:
  • 64 pelvic chondrosarcomas treated with resection
  • 5-year survival: 58% (worse than appendicular lesions)
  • Local recurrence: 35% (higher than appendicular lesions)
  • Adequate margins achieved in only 40% (due to anatomic constraints)
Clinical Implication: Pelvic location confers worse prognosis due to difficulty achieving adequate margins and late presentation.
Limitation: Retrospective study; heterogeneous resection types and reconstruction methods.

Clear Cell Chondrosarcoma: A Distinct Variant with Low-Grade Behavior

3
Bjornsson J, Unni KK, Dahlin DC • Human Pathology (1984)
Key Findings:
  • 35 clear cell chondrosarcomas from Mayo Clinic series
  • Epiphyseal location (unlike conventional chondrosarcoma)
  • Younger age (mean 38 years) vs conventional type (55 years)
  • Excellent prognosis: 15% local recurrence, under 5% metastases
  • Wide resection curative in majority of cases
Clinical Implication: Clear cell subtype has distinct clinical behavior - low-grade malignancy with excellent prognosis if adequately resected.
Limitation: Rare subtype (under 2% of chondrosarcomas) with limited long-term data; may be misdiagnosed as chondroblastoma.

Exam Viva Scenarios

Practice these scenarios to excel in your viva examination

VIVA SCENARIOStandard

Scenario 1: Distinguishing Enchondroma from Low-Grade Chondrosarcoma

EXAMINER

"A 55-year-old man presents with mild thigh pain for 6 months. X-ray shows a 6cm lobulated lesion in the proximal femoral metaphysis with rings and arcs calcification and endosteal scalloping involving two-thirds of the cortical thickness. What is your assessment and management?"

EXCEPTIONAL ANSWER
This presentation is concerning for a low-grade chondrosarcoma of the proximal femur. The key features suggesting malignancy are: age over 40 years, pain in a cartilage lesion, proximal long bone location (enchondromas favor hands), size greater than 5cm, and deep endosteal scalloping. I would take a systematic approach: First, complete staging with MRI of the entire femur to assess medullary extent, soft tissue extension, and joint invasion, plus CT chest to exclude metastases. Second, core needle biopsy along the lateral thigh in line with a definitive incision to confirm diagnosis and obtain histological grade. Third, discuss at multidisciplinary tumor board. If confirmed as chondrosarcoma, treatment is wide surgical resection with 1-2cm margins - intralesional curettage is contraindicated even for Grade I lesions. Reconstruction would likely be endoprosthesis or allograft-prosthetic composite depending on bone stock and patient factors. I would counsel about excellent prognosis with adequate resection (90% 10-year survival for Grade I), but emphasize that unlike osteosarcoma, chondrosarcoma is resistant to chemotherapy and radiotherapy so surgery is the only curative treatment.
KEY POINTS TO SCORE
Identify features distinguishing chondrosarcoma from enchondroma (PEST mnemonic)
Systematic staging: MRI entire bone, CT chest, biopsy
Emphasize wide resection is mandatory - no role for curettage
State that chondrosarcoma is chemo-resistant and radio-resistant
COMMON TRAPS
✗Recommending curettage for 'low-grade' lesion - incorrect, recurrence rate 50-90%
✗Missing need for MRI of entire femur (skip lesions, proximal extent)
✗Suggesting chemotherapy or radiotherapy as adjuvant - ineffective for conventional chondrosarcoma
LIKELY FOLLOW-UPS
"What imaging features differentiate enchondroma from chondrosarcoma?"
"What is the role of PET scan in chondrosarcoma?"
"How would your management differ if this were in the hand?"
VIVA SCENARIOChallenging

Scenario 2: Dedifferentiated Chondrosarcoma

EXAMINER

"A 65-year-old woman presents with severe hip pain and a large palpable mass. Imaging shows a large pelvic mass with two components: a mineralized chondroid lesion in the ilium plus an adjacent non-mineralized aggressive soft tissue mass. Biopsy shows low-grade cartilage with areas of high-grade pleomorphic sarcoma. What is your diagnosis and management?"

EXCEPTIONAL ANSWER
This is a dedifferentiated chondrosarcoma of the pelvis - the biphasic imaging and pathology are pathognomonic. Dedifferentiated chondrosarcoma is the worst prognosis subtype with only 7-24% 5-year survival. My management differs significantly from conventional chondrosarcoma: First, full staging including CT chest and PET-CT as metastatic rate is over 90% at time of death. Second, this is the ONLY chondrosarcoma subtype that receives chemotherapy - I would administer neoadjuvant chemotherapy with doxorubicin and ifosfamide to treat the high-grade non-cartilaginous component before surgery. Third, surgical planning with CT 3D reconstruction to determine resectability - this appears to be a Type I or II pelvic resection. Fourth, wide en bloc resection with negative margins if feasible, followed by adjuvant chemotherapy. Given the pelvic location and aggressive biology, achieving adequate margins is challenging and amputation (hemipelvectomy) may be required. I would counsel about very poor prognosis, high risk of lung metastases, and significant surgical morbidity. Radiotherapy may be considered for positive margins. Close surveillance every 2-3 months in first 2 years is essential.
KEY POINTS TO SCORE
Recognize biphasic pattern (cartilage plus high-grade sarcoma) as dedifferentiated chondrosarcoma
State this is the ONLY chondrosarcoma subtype receiving chemotherapy
Neoadjuvant chemotherapy before surgery (treat high-grade component)
Emphasize very poor prognosis (5-year survival 7-24%)
COMMON TRAPS
✗Treating as conventional chondrosarcoma without chemotherapy - incorrect
✗Missing the biphasic nature on imaging (chondroid plus non-mineralized aggressive mass)
✗Not recognizing pelvic location makes achieving margins very difficult
LIKELY FOLLOW-UPS
"What chemotherapy regimen would you use and why?"
"What are the surgical options for pelvic resection?"
"How would you reconstruct after Type II pelvic resection?"
VIVA SCENARIOCritical

Scenario 3: Positive Margins After Resection

EXAMINER

"You have resected a Grade II chondrosarcoma of the distal femur with endoprosthetic reconstruction. Final pathology reports positive margins posteriorly where tumor abuts the popliteal vessels. How do you manage this?"

EXCEPTIONAL ANSWER
Positive margins significantly increase local recurrence risk from under 10% to 40-60%, so this is a critical situation. I would take the following approach: First, discuss at multidisciplinary tumor board with oncology, radiology, and pathology. Second, assess the patient - are they fit for re-resection? Third, assess anatomical feasibility of re-resection - in this case, achieving negative margins posteriorly would likely require sacrifice of popliteal vessels, resulting in amputation. Fourth, discuss options with patient: Option A is re-resection (likely amputation) to achieve negative margins - this gives best chance of cure but loss of limb. Option B is adjuvant radiotherapy to the positive margin area - although chondrosarcoma is radio-resistant, high-dose radiotherapy (60-70 Gy) may provide some local control, accepting 40-60% recurrence risk. Option C is close surveillance and salvage amputation if local recurrence develops. I would counsel that re-resection with negative margins gives best oncological outcome, but given the significant functional loss (amputation), many patients choose radiotherapy plus close surveillance. Close imaging surveillance every 3 months for 2 years is mandatory regardless of choice. I would emphasize prevention - meticulous surgical planning and intraoperative frozen sections of margins can help avoid this situation.
KEY POINTS TO SCORE
Positive margins increase recurrence from 10% to 40-60%
Options: Re-resection, radiotherapy, or surveillance
Re-resection gives best oncological outcome but may require amputation
Involve patient in shared decision-making about function vs cure
COMMON TRAPS
✗Recommending chemotherapy for positive margins - ineffective in conventional chondrosarcoma
✗Not discussing amputation vs radiotherapy options with patient
✗Missing that this is a shared decision-making scenario balancing cure vs function
LIKELY FOLLOW-UPS
"What is the role of frozen section during resection?"
"What radiotherapy dose would you recommend for positive margins?"
"How would you counsel a young patient about amputation vs radiotherapy?"

MCQ Practice Points

Epidemiology Question

Q: Which statement about chondrosarcoma epidemiology is correct? A: Chondrosarcoma accounts for 20% of primary bone malignancies, making it the second most common after osteosarcoma. Peak age is 50-70 years with male predominance 2:1. Most common sites are pelvis (30-40%) and proximal femur (20-30%).

Pathology Question

Q: What is the most important prognostic factor in chondrosarcoma? A: Histological grade is the single most important prognostic factor. Grade I has 90% 10-year survival, Grade II 81%, Grade III 29%. Grade is more important than size or anatomic location in determining prognosis.

Treatment Resistance Question

Q: Why is chemotherapy not used for conventional chondrosarcoma? A: Conventional chondrosarcoma is resistant to both chemotherapy and radiotherapy due to poor vascularity of cartilage matrix and low cell turnover. Wide surgical resection is the ONLY curative treatment. Exception: Dedifferentiated and mesenchymal subtypes receive chemotherapy.

Subtype Question

Q: Which chondrosarcoma subtype has the worst prognosis? A: Dedifferentiated chondrosarcoma has the worst prognosis with only 7-24% 5-year survival. It shows biphasic pattern: low-grade cartilage plus high-grade non-cartilaginous sarcoma. Treated with neoadjuvant chemotherapy unlike conventional chondrosarcoma.

Imaging Question

Q: What imaging features help distinguish chondrosarcoma from enchondroma? A: Features suggesting malignancy (PEST mnemonic): Pain, Endosteal scalloping greater than two-thirds cortical thickness, Size greater than 5cm, Tracer uptake on bone scan or PET. Also: axial skeleton location, soft tissue mass, and age over 40 years.

Surgical Margins Question

Q: What is the local recurrence rate with positive margins after chondrosarcoma resection? A: Positive margins increase local recurrence from under 10% (negative margins) to 40-60% (positive margins). Margin status is the most important surgical factor for local control. Re-resection should be considered if anatomically feasible.

Australian Context and Multidisciplinary Management

Sarcoma MDT Referral

All chondrosarcomas must be discussed at Sarcoma MDT before biopsy and treatment.

  • CanTeen Sarcoma Unit (Queensland)
  • Peter MacCallum Cancer Centre (Victoria)
  • Chris O'Brien Lifehouse (NSW)
  • Royal Adelaide Hospital (SA)

Do NOT biopsy before MDT discussion - poorly planned biopsy compromises limb salvage.

Australian Sarcoma Registry

Bone Sarcoma Registry:

  • Voluntary submission of all bone sarcoma cases
  • Tracks outcomes, treatment protocols
  • Contributes to international collaborative trials

Medicolegal Considerations

Key documentation and consent points:

  • Diagnosis delay litigation: Pain in cartilage lesion requires investigation - document imaging and biopsy decisions
  • Biopsy tract contamination: Document MDT discussion and biopsy planning by sarcoma surgeon
  • Margin status: Intraoperative frozen sections, document decision-making if margins compromised
  • Amputation consent: Discuss possibility of amputation before limb salvage surgery if margins cannot be achieved
  • Prognosis discussion: Document discussion of grade-specific survival rates
  • Surveillance protocol: Provide written follow-up schedule and emphasize importance of compliance

Multidisciplinary Team Composition

Essential members:

  • Orthopaedic oncologist (surgeon)
  • Medical oncologist
  • Radiation oncologist
  • Musculoskeletal radiologist
  • Musculoskeletal pathologist
  • Specialist oncology nurse
  • Physiotherapist, occupational therapist
  • Psychologist, social worker

CHONDROSARCOMA

High-Yield Exam Summary

Key Pathology

  • •Second most common primary bone malignancy (20%)
  • •Malignant cartilage-forming tumor - rings and arcs calcification
  • •Histological grade is most important prognostic factor
  • •Grade I: 90% 10-year survival, Grade III: 29%
  • •Conventional type 85%, dedifferentiated worst prognosis (7-24% 5-year survival)

Clinical Presentation

  • •Pain in cartilage lesion (70-80%) - key symptom
  • •Pelvis 30-40%, proximal femur 20-30% - central (medullary) subtype
  • •Peak age 50-70 years, male predominance 2:1
  • •PEST features suggest malignancy: Pain, Endosteal scalloping, Size greater than 5cm, Tracer uptake

Imaging Protocol

  • •X-ray: Rings and arcs calcification, endosteal scalloping greater than two-thirds
  • •MRI entire bone: T2 high signal lobulated, assess extent and soft tissue invasion
  • •CT chest: Staging for lung metastases (90% of metastases)
  • •Core needle biopsy: Adequate tissue from most aggressive area (high T2, cortical destruction)

Treatment Principles

  • •Surgery is ONLY curative treatment - chemo-resistant and radio-resistant
  • •Wide resection with 1-2cm margins - intralesional curettage contraindicated
  • •Positive margins: 40-60% recurrence vs negative margins under 10%
  • •Dedifferentiated type: ONLY subtype receiving chemotherapy (neoadjuvant)
  • •Radiotherapy: Only for unresectable or positive margins (60-70 Gy)

Prognostic Factors

  • •Histological grade: Most important factor (Grade I 90%, Grade III 29% 10-year survival)
  • •Anatomic site: Pelvic worse than appendicular (30-60% vs 70-90% 5-year survival)
  • •Margin status: Negative margins essential (positive margins increase recurrence 10% to 40-60%)
  • •Subtypes: Periosteal best, dedifferentiated worst prognosis
Quick Stats
Reading Time140 min
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