Rhabdomyosarcoma radiotherapy indications and outcome

Rhabdomyosarcoma
Radiotherapy: Indications and Outcome
Víctor Barrondo
Radiation Oncology Department/Brachytherapy Section
Hospital Universitario Basurto, Bilbao

1

GEIS XIth International Symposium / Seville - November, 29th 2013

Intergroup Rhabdomyosarcoma Study

IRS I
IRS II
IRS III
IRS IV
COG D9602,D9803, D9802

(1972 – 1978)
(1978 – 1984)
(1984 – 1991)
(1991 – 1997)
(1998…)

OS 55%
OS 63%
OS 71%
OS 71%

3


4


Technology Costs
Before You get excited about the low
low price for 10Mb data storage, make
sure you read the ultra fine print. Turns
out the $3,495 price is for a refurbished
unit only. The new one retails for a
whopping $4,495. Oh, and there is
another catch: the price is in 1980
dollars (US). Adjusted to 2010 dollars,
that comes to around $13,395*
*US Per Capita personal Income

1980:

9,500$

2010:
40,500$
Source: U.S. Department of Commerce, Bureau of Economic
Analysis, Survey of Current Business.
http :// w w w. bea.gov/newsreleases/relsarchivespi.htm

5


Introduction

• Highly malignant neoplasm arising from embryonal mesenchyme
• With capacity for skeletal muscle differentiation.
• 40% of all soft tissue sarcomas and 7% childhood malignancies in the US

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Prognostic Factors

• Histology
• Stage
• Primary site (most important prognostic factor)
• Tumor size
• LN involvement (especially in extremities)
• Metastatic disease
• Group
• Extent of resection

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Intergroup Rhabdomyosarcoma Study Group Presurgical Staging System

Stage

Site

1

Orbit, Non PM H&N, GU non bladder/prostate, biliary tract

3

4

Regional Lymph Nodes

Distant Metastases

Any

N0, N1

M0

All Other Sites

≤5

N0

M0

All Other Sites

≤5

N1

M0

All Other Sites

2

Tumor Size (cm)

>5

N0, N1

M0

Any Site

Any Size

N0, N1

M1

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Intergroup Rhabdomyosarcoma Study Group Surgical-Patologic Grouping System

Group

Definition

I

Localized tumor, completely removed with patologically clear margins and no regional lymph node
involvement.

II

Localized tumor, grossly removed with (a) microscopically involved margins, (b) involved grossly resected
regional lymph nodes, or (c) both.

III

Localized tumor, with gross residual disease after grossly incomplete removal, or biopsy only.

IV

Distant metastases present at diagnosis

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Current Children’s Oncology Group (COG) Risk Groups

Histology

Group

Stage

Risk Group

ERMS

I, II, III

1

Low (Subset A)

ERMS

I, II

2, 3

Low (Subset B)

ERMS

III

2, 3

Intermediate

ARMS

I, II, III

1, 2, 3

Intermediate

ARMS

IV

4

High

ERMS

IV

4

High

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Radiotherapy Indications

The Short Answer is…
• Radiotherapy indicated in Group II-IV patients and Group I Alveolar histology.
• As a result from COG D9803, RT recommended at an early time point (4 Week).

.
Wolden SL, Anderson JR, Crist WM, et al. Indications for radiotherapy and chemotherapy after complete resection in
rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Studies I to III. J Clin Oncol 1999;17(11):3468e3475.
.
Raney RB, Anderson JR, Brown KL, et al. Treatment results for patients with localized, completely resected (group I) alveolar
rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984e1997: a report from the Children’s
Oncology Group. Pediatr Blood Cancer 2010;55(4):612e616.

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Radiotherapy Doses COG D9602/D9803
IRS- IV: Hyperfractionation (59.4Gy / 1.1Gy fraction b.i.d.) did NOT improve local,
regional or distant control over conventional fractionation for Group III tumors.

Microscopic Residual Disease
Resected Node Positive
Orbital Location Gross Disease
Gross Residual Disease
Second Look accepted

3600 cGy
4140 cGy
4500 cGy
5040 cGy

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Modern Radiotherapy Techniques: IMRT
• Early adopters since 1999.
• The next step in radiation treatment planning after 3D.
• Inverse planning with computer-assisted optimization.
• Dose painting.
Sharp dose fall off outside target volume with selective avoidance
of critical structures and tissues
• Multiple Fields.
Dose modulation within each field
• Better immobilization, longer treatment time.

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IMRT vs 3DCRT improved target dose coverage with no improvement in LC or FFS

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Smaller Margin: 15mm using MRI&PET fusion vs 20mm.
LFR 3 years: 5% Parameningeal & 0% Orbit/H&N.

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• Standard doses with PNI for adolescents
and young adults (n=21) and reduced doses
per fraction for children (n=20).
• 16%-18% (median, 17%) decrease in the
mean dose for rectum, bladder and bowel.
• 90%LC (100% children, 79% young adults).

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IMRT Functional Dose painting

A) Anatomical planning target volume (PTV).
B) Biological PTV.
C) Fusion of anatomical and biological PTV.
D) Contour based optimization.
E) Voxel signal intensity based optimization.


Modern Radiotherapy Techniques: Protons
• Inelastic collisions through matter
• Large proportion of the energy at the
Bragg peak and then falls off
• Particular advantage in H&N where
sparing of critical structures may be
better accomplished using protons,
particularly in young children.
• Long-term clinical outcomes comparing
proton therapy and IMRT not yet
available.
• Cost concern.

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Modern Radiotherapy Techniques: Protons

• LF 18% vs 17% in the IRSII-IV despite poor prognostic features compared with the
IRS trial population. (59% intracranial extension vs 38%) and median time to the
start of proton RT was 8 weeks (3/4LF late referral and ICE).
• Reduction in late effects compared with previously published series of patients
treated with photon RT (despite a higher proportion of younger patients).

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Favourable Sites
Orbit Rhabdomyosarcoma

•
•
•
•
•
•

Most commonly embryonal histology.
Small size.
Rarely lymph node involvement.
Standard treatment; QT+RT preserving eye.
3 year FFS 89%, OS 100%, 5 year LC 98%.
Toxicity: Cataract, keratopathy, dry eye, loss of vision is rare.

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Favourable Sites
Gynaecological Rhabdomyosarcoma

• Good prognosis.
• Common strategy: Initial QT with local therapy to remanent disease
(if present).
• Surgery rate decreased from IRS I-IV.
• Very good location for Brachytherapy.
• 5 year OS 82%.

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Favourable Sites
Paratesticular Rhabdomyosarcoma

• Initial management with Radical inguinal orchiectomy +/- RLND
(CT with enlarged nodes or 10 years or older patients).
• Avoid RT to scrotum to preserve contralateral testicular function if
complete resection achieved.
• 3 year FFS 90%.

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Unfavourable Sites
Extremity or Trunk Rhabdomyosarcoma

• High rate of lymph node involvement, node sampling required.
• Surgery maintaining form and function with RT preferred rather
than amputation.
• 10 year OS 63%, FFS 57%.

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Unfavourable Sites
Parameningeal Rhabdomyosarcoma

• Intracraneal extension of disease main prognostic factor.
• Surgery often limited (95% Group III).
• 5 year OS 73%, FFS 69%, LC 83%.

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Take Home Messages

• Close collaboration mandatory.
• Modern Radiotherapy techniques needed.
• Work Hard.

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GEIS
International Symposium / Seville - November, 29
ThankXIYou
th

th 2013

bitory2k@yahoo.com
@Bitorbar

Rhabdomyosarcoma radiotherapy indications and outcome

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