OSTEOSARCOMA

Moderators:
Prof. Shah Alam Khan Dr. Roshan Banjara
Dr. Arun Kumar Pandey
 INTRODUCTION
Primary malignant tumor of bone arising from primitive
bone-forming mesenchymal cells

L. Mirabello, R. J.Troisi, and S. A. Savage, “Osteosarcoma incidence and survival rates from 1973 to 2004: data
from the Surveillance, Epidemiology, and End Results Program,” Cancer, vol. 115, no. 7, pp. 1531–1543, 2009.
 SKELETAL DISTRIBUTION

AS – Anatomic Site
RS – Relative Survival
L. Mirabello, R. J.Troisi, and S. A. Savage, “Osteosarcoma incidence and survival rates from 1973 to 2004: data
from the Surveillance, Epidemiology, and End Results Program,” Cancer, vol. 115, no. 7, pp. 1531–1543, 2009.
 ETIOPATHOGENESIS

Savage SA, Mirabello L. Using Epidemiology and Genomics to Understand Osteosarcoma Etiology. Sarcoma.
2011;2011:548151
 CLINICAL FEATURES
Pain, Swelling
Loss of appetite
Weight loss
Chest pain/Cough

Tenderness
Local rise in temperature
Erythema
Dilated Veins
Movement at nearby joints
Distal neurovascular status
 INVESTIGATIONS – PLAIN X-RAY

Metaphyseal

Mixed pattern of
destruction
Codman triangle

Sunray/Sunburst
appearance
 INVESTIGATIONS – MRI
Extent of lesion
Resection margin
Soft tissue extension

Neurovascular
involvement
Joint involvement

Skip lesions
INVESTIGATIONS – BONE SCAN
INVESTIGATIONS - CT CHEST

Detect pulmonary
metastases
 BLOOD INVESTIGATIONS
Complete Haemogram

LDH

ALP

Liver and Renal Function

Tests
Positive correlation between Serum
Bone Alkaline Phosphatase and Lactate
Dehydrogenase with Tumor volume
 BIOPSY

Core Needle Biopsy preferred; Sensitivity: 93.1%,

Specificity:100% Positive and Negative predictive values of
100% and 99.9% respectively
Taupin T, Decouvelaere AV, Vaz G, et al.: Accuracy of core needle biopsy for the diagnosis of osteosarcoma: A
retrospective analysis of 73 patients. Diagn Interv Imaging. 2016; 97(3): 327–31.
 HISTOPATHOLOGY

Lichtenstein’s criteria:
Sarcomatous stroma
Spindle cells
Direct formation of
neoplastic osteoid and
bone
 STAGING

A system for the surgical staging of musculoskeletal sarcoma. 1980. Enneking WF, Spanier SS, Goodman MA. Clin
Orthop Relat Res. 2003 Oct;(415):4-18.
AJCC/UICC STAGING
 WHO CLASSIFICATION
PRIMARY OSTEOSARCOMA
CENTRAL (MEDULLARY)
CONVENTIONAL
TELANGIECTATIC
LOW GRADE INTRAMEDULLARY
SMALL CELL OSTEOSARCOMA
SURFACE (PERIPHERAL)
PAROSTEAL OSTEOSARCOMA
PERIOSTEAL OSTEOSARCOMA
HIGH GRADE SURFACE OSTEOSARCOMA
SECONDARY OSTEOSARCOMA
 CONVENTIONAL
OSTEOSARCOMA
High Grade Tumors

Intramedullary origin

Osteoblastic/Chondr
oblastic/Fibroblastic
Fibroblastic

Osteoblastic Chondroblastic
 TELANGIECTATIC
OSTEOSARCOMA
Purely lytic

Balloned
appearance
radiologically

Blood filled cyst

with very little
solid portion

. Matsuno T, Unni KK, McLeod RA, et al.Telangiectatic osteogenic sarcoma. Cancer. 1976;38:2538-2547.
LOW GRADE INTRAMEDULLARY
SARCOMA
Rare

Indolent course
With relatively
benign
radiographic
features

Mistaken for
Osteoblastoma/
Fibrous Dysplasia
 SMALL CELL OSTESARCOMA
Rare high
grade variant

Small blue cells

May resemble
Ewing Sarcoma/
Lymphoma

Sim FH, Unni KK, Beabout JW, et al. Osteosarcoma with small cells simulating Ewing’s tumor. J Bone Joint Surg Am. 1979;61:207-215.

Nakajima H, Sim FH, Bond JR, et al. Small cell osteosarcoma of bone: review of 72 Cases. Cancer. 1997;79:2095-2106.
PAROSTEAL OSTEOSARCOMA
Low grade

Lobulated ossified
mass on posterior
aspect of femur

Confused with
Osteochondromas
 PERIOSTEAL OSTESARCOMA

Intermediate Grade; Mostly arise from Diaphysis; Slightly

older age group ; Strands of osteoid producing Spindle
cells
 HIGH GRADE SURFACE
OSTEOSARCOMA
Least common

Aggressive

Histologically
similar
to Conventional
osteosarcoma

Medullary
involvement at
time of diagnosis
Surface osteosarcomas – distinct clinicopathological
entities

Upfront surgery

Better prognosis

Proper identifications
 SECONDARY OSTEOSARCOMA
Paget’s Disease: Benign pre-existing
conditions:
1% incidence
Osteochondroma
6th to 8th decade
Pelvis Bone infarcts

Previous Radiation Therapy: Fibrous dysplasia

1 % incidence in pts
treated with > 2500 cGy Chronic osteomyelitis
Unusual locations
Melorheostosis

Osteogenesis imperfecta
 DIFFERENTIAL DIAGNOSIS
Ewings sarcoma Metastasis

Chondrosarcoma Chronic Osteomyelitis

Fibrosarcoma Aneurysmal Bone Cyst

Osteoblastoma Chondroblastoma

Giant Cell Tumor

 MANAGEMENT
Primary Care
Physician

Orthopaedic Surgeon

Radiation Oncologist

Pathologist

Physiotherapist

Rehabilitation
specialist

Social workers
 TREATMENT OPTIONS

Chemotherapy

Surgery

Radiotherapy
CHEMOTHERAPY
Neoadjuvant Chemotherapy

Adjuvant Chemotherapy
 NEOADJUVANT
CHEMOTHERAPY
Reduction in tumor
volume

Response assessment

Immediate start

Time to prepare for

surgery
 CHEMOTHERAPEUTIC AGENTS

Multidrug regimen

MAP regimen gold standard

Alternative regimens
CHEMOTHERAPEUTIC REGIMEN
FOR OSTEOSARCOMA AT AIIMS
Doxorubicin and Cisplatin
3 cycles every 3 weeks

Surgery

Re-start 2-3 weeks post-

surgery
3 weekly x 40 weeks
 RE-STAGING AFTER NEO-
ADJUVANT CHEMOTHERAPY
Plain X-ray Indicators for
favourable response
MRI
 Tumor volume
FDG-PET Scan

 In angiographic
vascularity
mSUV – maximum Standardized uptake value, MTV – Metabolic Tumor Volume
 RADIOTHERAPY
Limited role

Pain relief in metastases

Inoperable sites

Lung irradiation

Schwarz R, Bruland O, Cassoni A, Schomberg P, Bielack S. The role of radiotherapy in oseosarcoma.

Cancer Treat Res. 2009;152:147-64.
 SURGERY
Definitive

Limb salvage
versus
amputation

Adequate
resection

Desirable salvaged
limb
Gitelis S , Malawer M , ,MacDonald D , Derman G . Chapman MW . Principles of limb salvage surgery , Chapman's
Orthopaedic Surgery , 2001 3rd edition Philadelphia, PA Lippincott Williams and Wilkins (pg. 3309 -3381)
 LIMB SALVAGE VERSUS
AMPUTATION
Major neurovascular involvement

Biopsy – Inappropriate/Complicated

Local infection/Fungation

Extensive soft tissue/muscle involvement

Pathological fracture (relative)

Local recurrence
Bacci G, Ferrari S, Lari S, Mercuri M, Donati D, Longhi A, Forni C, Bertoni F,Versari M, Pignotti E. Osteosarcoma of the limb.
 or limb salvage in patients treated by neoadjuvant chemotherapy. J Bone Joint Surg Br. 2002 Jan;84(1):88-92.
Amputation
APPROACH TO MANAGEMENT
Diagnosed Osteosarcoma

High
Assign surgical grade Neo-adjuvant
Assess tumor extent Grade Chemotherapy
Tumor

Plan surgical margins

Structures to be resected?
Probable functional losses?

Is salvage No
Amputation
advisable?
RESECTION OF TUMOR

Extraarticular

Intraarticular

Intercalary

Hemicortical

Simple
METHODS OF SKELETAL
RECONSTRUCTION
Resection and Simple reconstruction

Megaprosthesis

Expandable prosthesis

Osteoarticular Allografts

Alloprosthetic Composite

Resection arthrodesis

Rotationplasty
MEGAPROSTHESIS
MEGAPROSTHESIS
Average of 2.7 further operatios per
patient

Revision mainly for aseptic loosening

and infection
RESECTION AND SIMPLE RECONSTRUCTION
 INTERCALARY
AUTOGRAFT/ALLOGRAFT
ALLOPROSTHETIC COMPOSITE
ALLOPROSTHETIC COMPOSITE
EXPANDABLE PROSTHESIS
RESECTION ARTHRODESIS
ROTATIONPLASTY
PELVIC TUMORS
Iliofemoral and Ischiofemoral fusion
Surgical pseudoarthrosis(Mesh
reconstruction)
Pelvic allografts
Custom made endoprostheses
Saddle prostheses
Re-implantation of excised hemipelvis after
sterilization with radiation
9 patients with Osteosarcoma
5 patients with Ewing’s Sarcoma Family of
Tumors

2 year local recurrence free survival was 73 %

 PULMONARY METASTASIS
30% survival rate

Aggressive removal

Lung nodule metastasis until proven otherwise

 LOCAL RECURRENCE
Inadequate margins
Poor responders to chemotherapy

Poor survival following recurrence

Local recurrence-free survival 97% with
adequate surgical margins and 71 % with
inadequate surgical margins
Same was 95 % with good responders and 90
% with poor responders to chemotherapy
Initial tumor volume at presentation
does not predict local recurrence of
tumor
High Grade Older age
Increased ALP

Metastasis; Pulmonary versus

non-pulmonary
Axial location
Secondary
Osteosarcoma
Longer duration of symptoms

Normal BALP level

Good Performance status

 FUTURE DIRECTIONS
Chemotherapy – Individualization

Improved prostheses

Immunotherapy

Rotationplasty – Nostalgia ?
Correct order in decreasing frequency for
most common location of osteosarcoma is

1. Axial Skeleton > Distal femur > Proximal tibia >

Proximal Humerus
2. Proximal tibia > Distal femur > Axial Skeleton>
Proximal Humerus
3. Distal femur > Axial skeleton> Proximal tibia>
Proximal Humerus
4. Distal femur > Proximal tibia > Axial Skeleton>
Proximal Humerus
5. Distal femur > Proximal tibia > Proximal
Humerus> Axial Skeleton
 What is the single most important
parameter for prediction of local
recurrence of osteosarcoma ?

1. High Tumor Volume

2. Inadequate Surgical Margins

3. High Bone Alkaline Phosphatase

4. Presence of skip lesions

A 10 year old boy presents with pain over right thigh for 3
months, has raised serum LDH and BALP. MRI shows
extension of tumor to adjacent soft tissue. Biopsy shows
high grade osteosarcoma. CT scan chest shows no
pulmonary metastases, bone scan shows no metastases .
Correct stage as per Enneking staging System for this
osteosarcoma will be
1. IA
2. IB
3. IIA

4. IIB

5. III