INCTR

1
Retinoblastoma Strategy Group Report

• Annual Meeting 2004

2
Strategy Group History

• Initial Meeting INCTR Inaugural Meeting, 1999
  Problem Identification
• Late presentation, lack of professional
  awareness, treatment of advanced retinoblastoma
• 19 20th January, 2001
• To formalize study of reasons for late
  presentation
• To develop a strategic plan for improving early
  diagnosis
• To develop agreement in a common treatment
  protocol for this disease

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Strategy Group History

• 13 14th July, 2001
• To design and develop a common treatment protocol
  for patients with advanced disease
• 31st May, 2002
• To update group on the progress with the survey
  study Understanding Problems Faced by Parents of
  Children with Retinoblastoma Prior to Treatment
• To discuss the proposed treatment protocol

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Strategy Group History

• 29 May, 2003
• To update group on questionnaire study progress
• To finalize treatment protocol
• To discuss activities related to public and
  professional awareness campaigns

5
Strategy Group History

• 29 30th April, 2004
• To finalize the design of the treatment protocol
  for patients with extraocular retinoblastoma
• To review the results of questionnaire study and
  to discuss problems related to the questionnaire
• To revise the questionnaire and clinical data
  forms
• To provide updates on public and professional
  awareness campaigns

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Update on Public and Professional Awareness
Campaigns

• Brazil
• Televised Public Service Announcement
• Toll-free number and address provided for viewers
  to call or write in for advice
• Data on the number of cases diagnosed or referred
  is being kept (multiple hospitals/institutions
  providing data on cases diagnosed or referred)
• Telephone Card Campaign
• 1 million cards printed free and distributed to
  anyone purchasing a telephone care

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Update on Public and Professional Awareness
Campaigns

• Mexico
• Mexican Retinoblastoma Group
• Ophthalmologists and pediatric oncologists
  interested in retinoblastoma
• Studying cases of the disease
• Making publications about the pattern of disease
• Developing uniform treatment approaches
• Standardizing methods for improving early
  detection
• Poster Campaign
• TV Public Service Announcement

8
Update on Public and Professional Awareness
Campaigns

• India
• Professional Awareness for Ophthalmologists
• Presentations on chemoreduction
• International Meeting of Intraocular Tumors held
  in India exposing ophthalmologists to modern
  treatment approaches
• Public Awareness in India
• Full-length movie of the story of a child with
  bilateral retinoblastoma
• Best Feature Film of the Year in India
• Indian entry at Oscars

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Update on Public and Professional Awareness
Campaigns

• Turkey
• Retinoblastoma Day (highly publicized in
  newspapers)
• Improving early detection through professional
  education
• Coordinating the retinoblastoma treatment
  protocol within pediatric oncology in Turkey

10
Questionnaire Study Update

• Designed and initiated in 2001
• 15 institutions agreed to participate
  representing 11 countries
• 10 institutions have submitted data

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Questionnaire Study Update

• Institutions
• India - All India Institute of Medical Sciences,
  Tata Memorial Hospital, Cancer Institute Chennai
• Mexico - Instituto Nacional de Pediatria
• Turkey - Ankara University and Dokuz Euyul
  University
• Pakistan - Shaukhat Khanum Memorial Hospital
• Brazil Santa Marcelina Hospital
• Zimbabwe University of Zimbabwe
• Nigeria OAUTHC Obafemi Awolowo University

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Questionnaire Study Update

• Other Institutions
• Participated in Pilot
• Instituto Oncologico Del Oriente Boliviano,
  Bolivia
• Albert Einstein Hospital, Brazil
• Will Contribute in Near Future
• Philippines Childrens National Medical Center
• Ocean Road Cancer Institute, Tanzania

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Total Surveys Analyzed
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Age of Parents
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Children with Retinoblastoma

• Males/Females 57/43
• Indian institutions had more male patients when
  compared to other institutions (p0.015)
• Age
• Median Age 28 months
• Range 3 months to 15 years
• No significant differences in distribution of
  ages among institutions

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Family History of Retinoblastoma
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First Person to Notice a Problem
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Action Taken by Parents After First Symptom
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Who Parents Sought Advice From
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Actions Taken After Initial Advice
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Extent of Disease
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Association of Stage

• Fathers Education Level was associated with St
  Jude Stage
• Lower education level was associated with higher
  stages of disease (plt0.00005)
• Children from rural areas had higher stages of
  disease (p0.0064)

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Stage and Age at Diagnosis
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Stage and Time Interval (First Symptom to
Diagnosis)
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Fathers Education Level and Time Interval (First
Symptom to Diagnosis)
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Treatment Protocol

• Objectives
• To assess EFS, PFS and OS in patients with
  extraocular retinoblastoma treated with
  chemotherapy, radiation therapy and surgery, if
  needed
• To assess the toxicity of the protocol

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Treatment of Extraocular Retinoblastoma

• Eligibility Criteria
• Clinical, radiographic, histopathologic evidence
  of retinoblastoma
• Within 6 months of diagnosis
• May have had a prior enucleation
• Extraocular retinoblastoma as defined by soft
  tissues surrounding eye, the orbit, the sclera
  and/or optic nerve beyond the margin of resection

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Treatment of Extraocular Retinoblastoma

• Eligibility Criteria (continued)
• No evidence of extension of disease into meninges
  or CNS
• May have other distant metastatic sites such as
  bone marrow, lymph nodes
• Patients ages gt 9 months - lt 15 years
• Normal serum creatinine for age

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Treatment Protocol for Extraocular Retinoblastoma

• Treatment Schema
• Chemotherapy x 3 cycles
• Surgery (if needed)
• Chemotherapy x 3 cycles with concurrent radiation
  therapy
• Treatment Stratified by Optic Nerve Involvement

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Planned Activities

• Continuation of Public and Professional Awareness
  Campaigns
• Stimulate Activities within Country
• Finalization of the Treatment Protocol for
  Extraocular Retinoblastoma

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Retinoblastoma Strategy Group