Transcript Diapositiva 1

The International
Classification for
Retinoblastoma
Guillermo Chantada (1), Franois Doz (2), Celia Antoneli (3),
Richard Grundy (4), Clare Stannard (5), Ira J Dunkel (6),
Eric Grabowski (7), Carlos Leal-Leal (8), Carlos RodríguezGalindo (9), Enrique Schvartzman (1), Maja Beck Popovic (10),
Bernhard Kremens (11), Anna T. Meadows (12),
Jean-Michel Zucker(2)
(1)Hospital JP Garrahan, Hematology-Oncology, Buenos Aires, Argentina;
(2)Institut Curie, Pediatric Oncology, Paris, France; (3)Hospital AC Camargo,
Pediatric Oncology, Sao Paulo, Brazil; (4) Birmingham Children´s Hospital,
Pediatric Oncology, Birmingham, United Kingdom; (5) Groote Schuur Hospital and
University of Cape Town, Radiation Oncology, Cape Town, South Africa, (6)
Memorial Sloan Kettering Cancer Center, Pediatrics, New York, United States;
(7) Massachussetts General Hospital-Harvard Medical School, Boston,
Massachusetts, United States, (8) Instituto Nacional de Pediatría, Oncology,
Mexico, Mexico; (9) St Jude Children´s Research Hospital, Hematology-Oncology,
Memphis, United States; (10)’CHUV, Pediatric Hemato-Oncology Unit , Lausanne,
Switzerland; (11)University of Essen, Pediatric Oncology, Essen, Germany; (12)
Childrens Hospital of Philadelphia, Division of Oncology, Philadelphia, United
States
Publications from Latin America
(1995-2005)
• Argentina: Two prospective studies.
Grabowski-Abramson classification
• Brazil: Two prospective studies. CCG
classification
• Mexico: Retrospective study on 500
patients. St Jude’s Classification
Publications from developed
countries
• Latest prospective study: Howarth et al,
1980. St Jude’s classifcation
• France: Khelfaoui et al, 1996. No staging
info
• USA: Honnovar et al, 2002; Uusitalo et al
2001. No staging info
• Several countries: Autologous stem cell
transplantation: No staging info
Objectives
• To develop a new classification to
discriminate subgroups with different
survival
• To allow for comparison of different
centers
• To help discriminate between subgroups of
potential different outcome
There is no widely used staging
system for extraocular
retinoblastoma
Why?
Retinoblastoma classifications
• Grabowski-Abramson (Hematol Oncol Clin North
Am. 1:721-735,1987) updated by Abramson
Classification 2002
• St Jude´s (Cancer 1980, 45-851-858, updated
1997)
• TNM (UICC, latest version 2002)
• CCSG (Wolff et al, 1978)
• Cape Town. (Br J Ophthalmol 1979, 63,560-570)
updated 2002
Many include ophthalmological
data unfamiliar to the oncologist
• Included in St Jude’s classification
• Included in TNM classification
• Included in the Cape Town classification
Some do not consider all
prognostic factors
• CCG: No mention of postlaminar optic
nerve or choroidal invasion
• St Jude: No definition of choroidal
invasion, does not mention postlaminar
invasion
• Grabowski-Abramson: Does not
discriminate degrees of choroidal invasion
• TNM & Cape Town: No definition of
choroidal invasion
The International Classification for
Retinoblastoma
• Stage 0: Not enucleated patients
• Stage 1: Enucleated patients with
complete resected tumors
• Stage 2: Enucleated patients with
microscopical residual
• Stage 3: Regional disease
• Stage 4: Metastatic Disease (a) not CNS
involvement (b) CNS disease
Major features of this classification
• Separates conservatively treated patients
from enucleated and metastatic ones
• Proposes microstaging for putative risk
factors of enucleated eyes
• Extent of extraocular disease by imaging
studies and pathology (e.g. CSF and BM)
Risk stratification according to stage
Stage 0 and 1
Stage 2 and 3
Stage 4
Potential for cure
C
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Information needed for treatment
decisions
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Post laminar optic nerve extension
Choroid invasion
Scleral invasion
Other ocular coats involvement (anterior
segment)
• Combination of these features
Optic Nerve microstaging
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N0. No tumor in optic nerve
N1. Anterior lamina cribrosa
N2. Posterior lamina cribrosa
N3. Cut section and/or subarachnoid
invasion
• NX. Unknown
Choroid microstaging
• C0. No choroidal invasion
• C1. Superficial choroid invasion
• C2. Deep choroid invasion
Scleral Microstaging
• S0. No scleral involvement
• S1. Microscopical extension into sclera
• S2. Microscopical extension through
sclera into the orbit
What will be this classification used
for?
• Having a standard for eye pathology and
extent of disease evaluation
• Comparing among different groups
• Assessing incidence and disease
extension by international registries
• Defining the need for adjuvant therapy in
special subgroups
Challenges
• To define minimum standards for
pathological processing of enucleated
eyes
• To prospectively validate the classification
in a larger cohort
• To disseminate it to groups and centers
with high patient burden
Future steps
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Interest eye pathologists
Set up definitions
Set up a registry
Provide a facility for centralized review for
developing countries
• e-teaching support
• Analyze data to validate results