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EBRT IN EWINGS & OSTEOSARCOMA
Prarabdh Singh JR3
Akansha Anup SR1
FLOW OF SEMINAR
Epidemiology
Diagnostic staging & workup
Management :Chemotherapy & local therapy
RT Planning in Ewings sarcoma
EPIDEMIOLOGY
 2nd most common primary bone cancer
• 1 new case per 1 million population in US
• Between age 10-19 yrs incidence rises to 9-10 per million
• Whites affected twice as compared to Asians/Africans
• Male to female: 1.3:1
• Indian Incidence: Difficult to determine as rare and highly
underdiagnosed
S.J. Cotterill et al, JCO: 18;2000,
3108- 3114
Sites
• Locoregional Pain
• Palpable Mass
• Low grade Fever
• Site Specific Symptoms
• Metastatic Disease-Non Specific Symptoms(25% at
diagnosis)
• Median Duration Of Onset of Symptoms to definite
Diagnosis:3-9 months
CLINICAL FEATURES
WORKUP
• Routine blood inv(CBC,Biochem,Electrolytes)
• ESR
• LDH
• Radiology
• Primary Biopsy
• Bone Marrow Biopsy(BM spread in approx. 10% cases)
• Molecular Pathology/Genetics
Imaging
• X ray
• CT scan
• MRI
• Bone scan
• PET scan
PATHOLOGY
• Classic Ewing’s sarcoma appears as
sheets of monotonous round cells.
• Scanty cytoplasm & round nuclei with
evenly distributed finely granular
chromatin & inconspicuous nucleoli.
• Strong,diffuse membrane staining is
observed with the O13 monoclonal
antibody to p30/32MIC2 (CD99).
MANAGEMENT
Induction
chemo
Local RT/Sx
Maintainence
Chemo
12-24 Weeks VAC/IE AT 12/18weeks Upto 1year
CHEMOTHERAPY
EFT 2001 protocol(TMH)
LOCAL THERAPY
• Surgery Radiotherapy
• Types of resection: Types:
I. Intralesional Pre-op
II. Marginal Definitive
III. Wide excison Post-op
IV. Radical
Safety margin -atleast
Bone-1cm margin
Muscle/fat-5mm margin
Fascia-2mm margin
RADIOTHERAPY
Indications of Radiotherapy
Definitive
Tumors where Resection
is Impossible
For skull, face, vertebra,
or pelvic primary
where only an intra-
lesional resection is
achievable
Patient with poor
Surgical risk
Patient refusing surgery
Preoperative
Narrow resection
margin expected
Sterilize tumor
compartment
Reduce risk of
dissemination
Postoperative
For gross or microscopic
positive margin
For marginal Resection
For wide-resection with
Poor Histological
response to Neo-
adjuvant Chemotherapy
(>10% viable
tumor cells in the
specimen)
Based on CESS-81, CESS-86, EICESS-92 Studies : Schuck et al,IJROBP-1998 & 2003
• Gross tumor- 55-60 Gy
• Microscopic Residual Disease-50 -55 Gy
• No residual Disease(Post op poor response to chemo)-45-50 Gy
Target:
The appropriate irradiated volume is an involved field to the pretreatment tumor
volume plus 2-2.5cm margin, followed by a boost to the post-induction
chemotherapy tumor volume with margin
https://doi.org/10.1002/pbc.10472
Laskar S ,Mallick I. et al. Pediatr Blood Cancer 2008;51:575–
580
In an analysis of patients receiving PORT in the CESS 86
and EICESS trials,
Schuck et al
no significant difference in the local control and survival
who received RT within 60 days of surgery or later.
Dunst J,et al -
improved local control in CESS 86 over CESS 81
timing of RT was brought forward from the 18th week to the 10th week
Dunst J, Results of CESS 81 and CESS 86. Cancer 1991;67:2818–2825
Schuck A,. Strahlenther Onkol 2002;178:25–31.
Timing of Post-Operative Radiation
FIG. Changes in treatment volume. (A) Field
encompassing the entire length of the medullary cavity
for a tumor involving the proximal left humerus. (B)
Tailored field encompassing only the proximal aspect of
the leg for a limited tumor of the left tibia.
STEPS OF PLANNING
Positioning &
Immobilization
Planning
CT Scan
Target
Volumes
Delineation
Treatment
Planning
Process
CASE CAPSULE
15 years female
P.w.c.o pain in right buttock region radiating to right leg since july 2019
Difficulty in walking since sept 2019
Investigations:
PETCECT(25.5.2020)
FDG avid STM involving sacrum & coccyx with erosive lesion
Low FDG avid mesentric LN anterior to right kidney
Sacrum mass bx (1.6.20)
Ewings sarcoma
Bone marrow: Uninvolved
Received 3# VIDE from 18.6.20 to L.D 25.7.20
PETCECT (2.8.20)
Low grade FDG uptake in lytic changes with STM
Plan:Definitive CTRT
Pre-chemo PET-CECT
Post Chemo Scan
POSITIONING
Head first supine Arms overhead, No Knee rest.
Abdo BP, 4 clamp Abdo orfit. All Outer clamps.
Fiducials placed at Pubic symphysis level
5 mm NCCT cuts taken.
Pushed to PACS.
PLANNING SCAN
CONTOURING
PLANNING
Dose Prescription:55.8Gy/31#
Technique:IMRT with IGRT
PLAN:CONVENTIONAL VS 3DCRT
 95% Isodose volume  95% isodose volume
PLAN:CONVENTIONAL VS 3DCRT
50% Isodose volume 50% Isodose volume
PLAN:CONVENTIONAL VS IMRT
95% isodose volume 95% Isodose volume
PLAN: CONVENTIONAL VS IMRT
50% Isodose volume 50% Isodose volume
PLAN:3DCRT VS IMRT
95% isodose volume 95% isodose volume
PLAN:3DCRT VS IMRT
50% Isodose volume 50% Isodose volume
PLAN :3DCRT VS IMRT
70% Isodose volume 70% Isodose volume
DVH
OAR:DVH(BLADDER)
DVH(RECTUM)
Structure Conventional 3DCRT IMRT
PTV V95%-96% 97% 96%
Bladder 55Gy 25.91Gy 23Gy
Rectum 52Gy 38.3Gy 36.1Gy
Bowel Bag 22Gy 11.4Gy 9.1Gy
Rt Femoral head 10.4Gy 7.9Gy 4.8Gy
Left femoral head 5.1Gy 3.8Gy 3.1Gy
CASE CAPSULE-2
12 year female
P.w.c.o swelling in forearm since 4 month
X ray Forearm(A+P):Diaphyseal expansile lesion in left leg
Bx :Ewings sarcoma
Started with EFT 2001-Good response
U/w I/C Fibulectomy on 5.6.20
HPR:88%necrosis,all margins free
Planned for Post Op RT
POSITIONING
 Supine ,arms by side ,Leg externally rotated
 Thermoplastic mask
CONTOURING
PLANNING
Dose prescription to tumor bed :45Gy/25#
Lung bath:12.6Gy/7#
LUNG BATH
Description:12.6Gy/7#
PROTON THERAPY
Retrospective review of 30 patients treated with proton therapy
between april 2003-April 2009 at Massachusetts hospital
14Male & 16female patients
Median dose:54Gy
Median F/u:38.4months
https://doi.org/10.1016/j.ijrobp.2011.03.038
3year actuarial rates
EFS 60%
LC 86%
OS 89%
FOLLOWUP
After completion of therapy all children shall be followed
up:
every 3 monthly for the first year
every 6 monthly for the 2nd & 3rd year
yearly thereafter
This is applicable to all patients except when
specified
Second malignant neoplasms
Radiation Induced Sarcoma
dose related
much less frequent < 48 Gy
Alkylator Induced Leukemia
alkylator dose more important than the addition of etoposide
Adriamycin cardiomyopathy
Ifosfamide: renal toxicity
Gonadal failure
Chronic pain/musculoskeletal dysfunction related to local therapies
Psychological Trauma - adolescents
Late Effects in ESFT Survivors
OSTEOSARCOMA
• Malignant mesenchymal tumour
• Characterized by production of osteoid matrix and woven
bone by tumor
• Molecularly:
• It is a sporadic complex genotype sarcoma, (as
distinguished from the balanced translocation- associated
sarcomas (e.g., Ewing sarcoma)): Cell cycle regulators such
as p53 and Rb have been implicated
(Li-Fraumeni, Hereditary Retinoblastoma
INTRODUCTION
EPIDEMIOLOGY
 Most commonly diagnosed primary malignancy of bone (Rare tumor
nonetheless
 Bimodal peak of incidence: One around adolescence and another
smaller peak in elderly (60+)
 Males affected slightly more frequently than females.
•Malignant spindle cells
•Osteoblasts
•Osteoid/immature bone matrix
Pathologic Classification of tumor
•METASTASIS:
•Disseminate through blood
•1st site – lung 9.3%
•2nd bone 3.9%
•Lymph node involvement only <10% -poor prognostic sign.
•SKIP METASTATIS:
•Located within the same bone as the main tumour but not in continuity with it.
•High grade sarcomas-develop by embolisation of tumour cells within the marrow sinusoids—
represent local micromets
•Clinical incidence -1%. Predict poor prognosis
•Site
•Pain and soft tissue swelling
•Night pain, no effusion in adjacent joint and movements are normal.
•Age > 40 yrs – pre existing disease
•Paget’s disease, irradiation, multiple hereditary exostosis, polyostotic fibrous
dysplasia.
•Associated syndromes-
•Li fraumeni syndrome
•Rothmund Thomson syndrome
•Werner’s syndrome
•CENTRAL/INTRAMEDULLARY(90-95%)
•CLASSIC(CONVENTIONAL) 75-80%-HIGH GRADE
•OSTEOBLASTIC
•CHONDROBLASTIC
•FIBROBLASTIC
•LOW GRADE
•SMALL CELL
•TELANGIECTATIC
•SECONDARILY IN ABNORMAL BONE(PAGET’S)
•SURFACE (5-10%)
•PAROSTEAL-LOW GRADE
•PAROSTEAL-HIGH GRADE
•PERIOSTEAL
•EXTRAOSSEOUS
IMAGING
X-RAY
 CT SCAN
 MRI
 BONE SCAN
 ANGIOGRAPHY
Definitive Indications
Unresectable tumor
Preoperative Indications
Adjuvant Indication
• Margin+
• Head & Neck Cancer
DOSE
Definitive setting:70.2Gy/39#
Adjuvant settig:64.8Gy/36#
CASE CAPSULE -1
(DEFINITIVE)
21yrs,male ,Hailing from kolkata
p.w.c.o left anterior & lateral chest wall pain X5months
Investigations:
1)MRI Thorax
11x5.2cm lobulated chest wallmass in left & posterior chest wall with
encasement of 3rd & 6th rib underlying nodular extrapleural and overlying
extraosseous soft
tissue components at the intercostal muscles
Receive 6# of chemo
2)CT Guided bx :Chondroblastic OGS
Plan:Definitive RT
Pre-Chemo Scan
Post chemo response assesment
CONTOURING
PLANNING
DOSE PRESCRIPTION:70.2GY/39#
CONVENTIONAL VS 3DCRT
95% Isodose volume 95% Isodose volume
CONVENTIONAL VERSUS 3DCRT
50% Isodose Volume
50% Isodose Volume
CONVENTIONAL VS IMRT
95% OF ISODOSE VOLUME 95% OF ISODOSE VOLUME
CONVENTIONAL VS IMRT
50% Isodose volume 50% Isodose volume
DVH(COMPARISON)
Structure Conv 3DCRT IMRT
PTV V95-96% V95-96% V95-97%
Lt Lung 43.9Gy 28.45Gy 22.1Gy
Rt Lung 1.5Gy 5.5Gy 4.8Gy
Heart 3.9Gy 2.1Gy 1.5Gy
Spinal cord 65Gy 60Gy 44.5Gy
CASE CAPSULE-2 (POST OP)
26 Year Female from Lucknow
H/o nasal obstruction & epistaxis in march 20
MRI 15.10.20 :Lobulated lesion in right nasal cavity with intraorbital
extension
U/w FESS +Bx--.Osteosarcoma
Received 4# Cis adria
MRI H+N:
Residual lesion
U/w endoassisted resection of sinonasal osteosarcoma
Plan:Adjuvant RT
IMAGING
Post op MRI
CONTOURING
PLANNING
Dose prescription: 63Gy/35#
Technique:IMRT with IGRT technique
CONVENTIONAL VS 3DCRT
50% Isodose volume 50% Isodose volume
CONVENTIONAL VS 3DCRT
95% Isodose volume 95% Isodose volume
CONVENTIONAL VS IMRT
95% Isodose volume 95% Isodose volume
CONVENTIONAL VS IMRT
50% Isodose volume 50% Isodose volume
DVH(COMPARISON)
Structure CONV 3DCRT IMRT
PTV V95%-93% 91% 92%
Left eye 66.53Gy 26Gy 10.5Gy
Right eye 23.1Gy 35Gy 22Gy
Chiasma 45.8Gy 28Gy 23Gy
Left optic nerve 28.5Gy 26.1Gy 10.5Gy
Right optic nerve 62Gy 57Gy 22.2Gy
THANK YOU

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