OSTEOSARCOMA

Moderators:
Prof. Shah Alam Khan Dr. Roshan
Dr. Arun Kumar Banjara
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. 15311543, 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. 15311543, 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
 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): 32731.
 HISTOPATHOLOGY

Lichtensteins
criteria:
Sarcomatous
stroma
Spindle cells
Direct formation of
neoplastic osteoid
and bone
 STAGING

Asystemfor thesurgicalstagingofmusculoskeletalsarcoma. 1980. EnnekingWF,

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/Chond
roblastic/Fibroblasti Fibroblastic
c

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 Ewings 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
Osteochondrom
as
 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
Pagets Disease: Benign pre-existing
conditions:
1% incidence
Osteochondroma
6th to 8th decade
Pelvis Bone infarcts

Previous Radiation Fibrous dysplasia

Therapy:
1 % incidence in pts Chronic osteomyelitis
treated with > 2500
cGy Melorheostosis
Unusual locations
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
MRI response

FDG-PET Scan Tumor volume

In angiographic
vascularity
mSUV maximum Standardized uptake value, MTV Metabolic
 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. Amputation or limb salvage in patients treated by neoadjuvant
chemotherapy. J Bone Joint Surg Br. 2002 Jan;84(1):88-92.
APPROACH TO MANAGEMENT
Diagnosed Osteosarcoma

High
Assign surgical grade Neo-adjuvant
Assess tumor extent Grad Chemotherapy
e
Tumo
Plan surgical margins r
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 Ewings 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 ?
T
H
A
N
K
Y
O
U
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