Clinical Approaches to Early Detection and Management of Retinoblastoma

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Clinical Approaches to Early Detection and
Management of Retinoblastoma
Francis Munier Aubin Balmer Jules Gonin Eye
Hospital (Prof. Leonidas Zografos) Lausanne,
Switzerland
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Retinoblastoma

• Hereditary retinoblastoma (40) unilateral or
  bilateral, sporadic or familial (mean age at
  diagnosis 12 m)
• Non hereditary retinoblastoma (60) Always
  unilateral and sporadic (mean age at
  diagnosis 24 m)

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Fig 12.6
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Fig 12.8
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Gene Directed Management pre- or
neonatal molecular diagnosis

• Surveillance under anesthesia
• Presymptomatic diagnosis
• Conservative treatment

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Retinoblastoma frequency

• 17 of neonatal cancers
• 6.5 of pediatric cancers 1/20000 live birth
• 10 of ocular cancers

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First sign of retinoblastoma

• Leucocoria (60)
• Strabismus (20)
• Aypical manifestations (20)

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Fig 12.17
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Fig12.17 d,e
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Fig 12.19abc
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Ivelin (dia à scanner)
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Is retinoblastoma a recent pathology in medical
history?

• Peter Pavius April 5th 1597

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Fig. 12.1
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Fig 12.2
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12.4
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Fig 12.3a
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Retinoblastoma 5 year survival
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Therapeutic modalities

• Enucleation
• Conservative treatments

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Importance of tumor staging by the ophthalmologist

• ABC classification of Murphree for intra-ocular
  retinoblastoma

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ABC Classification of Murphree

• Group A tumors lt 3 mm in hight, lt 3 mm from
  macula and/or optic nerve head, no VS, no RD
• Group B tumors gt 3 mm in hight, RD lt 5 mm
  around tumor base, no VS, no subretinal
  seeding
• Group C localized or fine diffuse VS and/or RD
  gt 5 mm around tumor base (no subretinal
  growth)

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ABC Classification of Murphree

• Group D Massive VS (avascular growth) and/or
  RD with subretinal growth
• Group E No visual potential, neovascular
  glaucoma, tumor in the anterior segment, tumor
  anterior to the hyaloid, orbital cellulitis,
  buphthalmos

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Vue générale salle dop avec enfant
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Enucleation procedure

• Enucleation and resection of at least 10
  mm of optic nerve
• Reconstruction with Hydroxyapatite or Medpor
  orbital implant

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il noir et blanc énuclée
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Fig 12.151
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Fig 12.150
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Therapeutic modalities Whole-eye treatments

• External beam radiation with/without
  lens-sparing
• Chemoreduction

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External beam radiation

• 50 Gy with daily fraction of 1.8 Gy

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Schéma radiothérapie bleu
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Enfant sous chimiott (à scanner)
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Kaziu avant
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Kaziu après
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Side effects of EBR

• Ocular complications (cataract, maculopathy)
• Mid-facial growth defects
• Non-ocular second neoplasms

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Therapeutic modalities Whole-eye treatments

• External beam radiation with/without lens-sparing
• Chemoreduction

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Chemoreduction

• Cryorupture of the hemato-retinal barrier 24-48
  hours before infusion
• Surgical implantation of a Portacath
• Infusion of Carboplatin and VP16 D1-3
• 2-5 monthly cycles

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Dispositif chimio portacath (à scanner)
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Freiermuth avant
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Freiermuth après
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Freiermuth après
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Therapeutic modalities Focal treatments

• Cryocoagulation with/without conjunctival
  incision
• Transpupillary or transcleral photocoagulation
  (Xenon, Diode)
• Brachytherapy (Ruthenium, Iode)
• Hyperthermia, Thermochemotherapy
• Conformal stereotactic radiotherapy
• Proton beam irradiation

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Therapeutic modalities Focal treatments

• Cryocoagulation with/without conjunctival
  incision
• Transpupillary or transcleral photocoagulation
  (Xenon, Diode)
• Brachytherapy (Ruthenium, Iode)
• Hyperthermia, Thermochemotherapy
• Conformal stereotactic radiotherapy
• Proton beam irradiation

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Série laser transcléral 2
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Série laser transcléral 3
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Série laser transcléral 4
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Série laser transcléral 5
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Série laser transcléral 6
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Série laser transcléral 7
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Therapeutic modalities Focal treatments

• Cryocoagulation with/without conjunctival
  incision
• Transpupillary or transcleral photocoagulation
  (Xenon, Diode)
• Brachytherapy (Ruthenium, Iode)
• Hyperthermia, Thermochemotherapy
• Conformal stereotactic radiotherapy
• Proton beam irradiation

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Brachythérapie fig 12.156ab
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Fig 2.29
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Brachythérapie Bonelli à la pause
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Brachythérapie Bonelli suivi
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Therapeutic modalities Focal treatments

• Cryocoagulation with/without conjunctival
  incision
• Transpupillary or transcleral photocoagulation
  (Xenon, Diode)
• Brachytherapy (Ruthenium, Iode)
• Hyperthermia, Thermochemotherapy
• Conformal stereotactic radiotherapy
• Proton beam irradiation

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Thermochemotherapy

• Infusion of a single dose of Carboplatin 1-3
  hours before hyperthermia
• Transpupillary hyperthermia mediated by a diode
  laser on D1
• Second round of hyperthermia between D4 and D7
• 2-4 monthly cycles

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Schéma de focalisation sur la tumeur (à
scanner)
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Therapeutic modalities Focal treatments

• Cryocoagulation with/without conjunctival
  incision
• Transpupillary or transcleral photocoagulation
  (Xenon, Diode)
• Brachytherapy (Ruthenium, Iode)
• Hyperthermia, Thermochemotherapy
• Conformal stereotactic radiotherapy
• Proton beam irradiation

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Therapeutic modalities Focal treatments

• Cryocoagulation with/without conjunctival
  incision
• Transpupillary or transcleral photocoagulation
  (Xenon, Diode)
• Brachytherapy (Ruthenium, Iode)
• Hyperthermia, Thermochemotherapy
• Conformal stereotactic radiotherapy
• Proton beam irradiation

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Fig 2.36
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fig. 2.38
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Fig 2.39 a
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Spot Scan technique Paul Scherrer
InstituteSwitzerland
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Proton Therapy Program
OPTIS for children a project for the future
A.G., 4 years , proton therapy for a relapsing
retinoblastoma in the left eye. Authorized
picture showing the child 18 months after
successful treatment.
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The retinoblastoma multidisciplinary team

• Pediatric oncologist Dr Maja Nenadov-Bec
• Radio-oncologists Dr Alessia Picca Raphaël
  Moeckli
• Pediatric anesthesists Drs Michèle Pot
  Olivier Poinsot
• Medical geneticist Prof. Daniel Schorderet
• Pathologist Dr Sylvie Uffer

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The retinoblastoma multidisciplinary team

• Secretaries Luisa de Medici Maria Spagnuolo
• Ocularist Dominique Charles-Messance
• Instrumentalists Brita Hudepohl et al.
• Social worker Ursula Figueiredo
• Aumonerie Emmanuel Alfani
• Photography Marc Curchod
• Data manager Sue Jaquet

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Les rameurs