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The International Classification for Retinoblastoma

This publication highlights the development of a new classification system for retinoblastoma to distinguish subgroups with varying survival rates and improve comparison among different centers. It aims to assist in determining the potential outcomes for different subgroups and standardizing the evaluation of disease extension.

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The International Classification for Retinoblastoma

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  1. The International Classification for Retinoblastoma

  2. 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 Rodríguez-Galindo (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

  3. 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

  4. 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

  5. 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

  6. There is no widely used staging system for extraocular retinoblastoma Why?

  7. 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

  8. Many include ophthalmological data unfamiliar to the oncologist • Included in St Jude’s classification • Included in TNM classification • Included in the Cape Town classification

  9. 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

  10. 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

  11. 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)

  12. Risk stratification according to stage Potential for cure Curability Stage 0 and 1 Stage 2 and 3 Stage 4

  13. Information needed for treatment decisions • Post laminar optic nerve extension • Choroid invasion • Scleral invasion • Other ocular coats involvement (anterior segment) • Combination of these features

  14. Optic Nerve microstaging • N0. No tumor in optic nerve • N1. Anterior lamina cribrosa • N2. Posterior lamina cribrosa • N3. Cut section and/or subarachnoid invasion • NX. Unknown

  15. Choroid microstaging • C0. No choroidal invasion • C1. Superficial choroid invasion • C2. Deep choroid invasion

  16. Scleral Microstaging • S0. No scleral involvement • S1. Microscopical extension into sclera • S2. Microscopical extension through sclera into the orbit

  17. 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

  18. 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

  19. Future steps • 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

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