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RETINOBLASTOMA
By
Dr. Ayush Garg
Clinical Anatomy
 The eye is composed of three
layers.
 Outer fibrous layer formed by the
sclera posteriorly and the cornea
anteriorly.
 Inner layer , sensory retina with
vision concentrated at the fovea
which is lateral to the optic nerve
and directly posterior to the lens.
 In between these vascular layer –
the uvea or choroid –which
supplies the retina. The iris is the
outer continuation of the vascular
layer
 Lens sits just behind iris,
suspended from the ciliary body.
 No lymphatic drainage
PATHOLOGY Flexner Wintersteiner
rosettes
-columnar cells around a
central lumen
-also seen in
medulloepithelioma
Homer Wright
-rosettes around a central
neuromuscular core
-neuroblastoma,
medulloepithelioma,
medulloblastomas
Fleurettes
Tumor cells with pear shaped
eosinophilic processes
projecting through
LEUKOCORIA
FUNDOSCOPIC EXAMINATION
Typical appearance of intraretinal retinoblastoma. Opaque, yellow-white
macular tumor fed and drained by dilated, tortuous retinal blood vessels.
RETINOMA-spontaneously arrested retinoblastoma
Limited vascularity
Greyish-white
Speckled
Surrounding chorio-retinal atrophy
RPE hypertrophy
A computed tomography scan of a large calcified retinoblastoma in the right eye.
Transaxial T2-weighted (TR/TE, 3,460/116 ms) (a) and
T1-weighted (TR/TE, 374/14 ms) precontrast (b) and
postcontrast (c)
MRI of exophytically growing retinoblastoma with secondary retinal
detachment. Retinoblastoma typically has low signal intensity
compared to the vitreous body on T2-weighted images and
intermediate signal intensity on precontrast T1-weighted images,
and it demonstrates marked contrast enhancement
ROUTES OF SPREAD
ORBITAL IMPLANT
RADIOACTIVE PLAQUE APPLICATION
Procedure: 1st USG of eye done: for
tumor dimensions: maximum basal
diameter, max height → surgical
exploration → applicators are applied
over sclera overlying the tumor → 1.5-2
mm margin on either side of basal
diameter → retention sutures → Rx →
Re-exploration for removal of plaques
Iodine 125 plaques
Ruthenium
plaque
sutured to
sclera
PATTERNS OF GROWTH
Radiotherapy
External beam radiotherapy
Indications
Multi-focal retinoblastoma
RB close to macula or optic nerve
Large tumor with vitreous seeding
Positioning
Proper immobilization is important
thermoplastic or Perspex shell
with the patient supine and the
chin in a neutral position
Treatment is done under
anesthesia: Ketamine
Energy: Co-60 or 4-6 MV photons
Technique
Lateral field technique
Started in 1930’s
D – shaped lateral field with anterior
border kept at bony orbit
Disadvantage: tumor recurrence at or
near ora serrata
Modified lateral beam technique
Two lateral opposed D-shaped fields
are used
Direct Anterior Field [Hungerford et al ]
Whole eye is treated
Disadvantage:
Cataract formation
Dose exits through brain
Lacrimal gland irradiation-impaired tear
production
Advantages:
Simple, easy to setup
Reproducible
Homogenously irradiates entire retina
Anterior lens sparing technique (ALD) [Abramson et al ]
Lateral D-shaped field: Day1-Day 4 by photons
Anterior electron beam field with central circular contact lens as lens
shaped shield: on Day 5
Unilateral disease:
one lateral field or
2 oblique portals [superior and
inferior]
Bilateral disease:
parallel opposed lateral fields
The anterior beam edge is
placed at the bony canthus
and the beam is angled 1.5
degrees posteriorly if the
contralateral eye remains in
place.
Dose
Ideal: 40-45 Gy, 1.8-2 Gy per fraction, 5 days a week
For large tumor /vitreous seeding: 48-50 Gy
Palliative radiotherapy:
Extra-ocular involvement: 20-25 Gy
Metastatic disease: CNS, bones: 5 Gy/1#, 15 Gy/3#
Unilateral : single lateral field
With anterior extension: anterior field + lateral field tilted 5-15°
posteriorly
Bilateral disease: parallel opposed lateral fields
3D CONFORMAL RADIOTHERAPY TECHNIQUE
Based on 3D CT scan planning
In unilateral RB, 4 non-coplanar fields are used.
fields are anterior oblique: superior, inferior, medial, and lateral.
0.5-cm bolus can be used.
entire retina should be treated, including 5 to 8 mm of the proximal optic
nerve.
Critical structures such as the opposite eye, optic chiasm, pituitary
gland, brainstem, posteriormost upper teeth, and upper cervical spine.
The tumor volume is treated to the 98% or 95% line,with the
aforementioned organs and tissues receiving significantly less dose.
bilateral disease, six
noncoplanar fields are used:
two lateral opposing, and
two anterior oblique fields
to each eye following the
same criteria described
previously.
3D CT scan reconstruction image showing beam arrangement for unilateral
RB : anterior medial and lateral fields (A), anterior superior and inferior fields
(B), sagittal view of composite isodose distribution (C), and axial transverse
view of isodose distribution (D)
Follow up
 1st 6 months: 4-6 week intervals
 Upto 3 years: at 4-6 month intervals
 Later, yearly
 Family history positive: All family members [other
children at birth] should be examined yearly
THANK YOU

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Retinoblastoma

  • 2. Clinical Anatomy  The eye is composed of three layers.  Outer fibrous layer formed by the sclera posteriorly and the cornea anteriorly.  Inner layer , sensory retina with vision concentrated at the fovea which is lateral to the optic nerve and directly posterior to the lens.  In between these vascular layer – the uvea or choroid –which supplies the retina. The iris is the outer continuation of the vascular layer  Lens sits just behind iris, suspended from the ciliary body.  No lymphatic drainage
  • 3. PATHOLOGY Flexner Wintersteiner rosettes -columnar cells around a central lumen -also seen in medulloepithelioma Homer Wright -rosettes around a central neuromuscular core -neuroblastoma, medulloepithelioma, medulloblastomas Fleurettes Tumor cells with pear shaped eosinophilic processes projecting through
  • 5. FUNDOSCOPIC EXAMINATION Typical appearance of intraretinal retinoblastoma. Opaque, yellow-white macular tumor fed and drained by dilated, tortuous retinal blood vessels.
  • 6. RETINOMA-spontaneously arrested retinoblastoma Limited vascularity Greyish-white Speckled Surrounding chorio-retinal atrophy RPE hypertrophy
  • 7. A computed tomography scan of a large calcified retinoblastoma in the right eye.
  • 8. Transaxial T2-weighted (TR/TE, 3,460/116 ms) (a) and T1-weighted (TR/TE, 374/14 ms) precontrast (b) and postcontrast (c) MRI of exophytically growing retinoblastoma with secondary retinal detachment. Retinoblastoma typically has low signal intensity compared to the vitreous body on T2-weighted images and intermediate signal intensity on precontrast T1-weighted images, and it demonstrates marked contrast enhancement
  • 10.
  • 11.
  • 12.
  • 14. RADIOACTIVE PLAQUE APPLICATION Procedure: 1st USG of eye done: for tumor dimensions: maximum basal diameter, max height → surgical exploration → applicators are applied over sclera overlying the tumor → 1.5-2 mm margin on either side of basal diameter → retention sutures → Rx → Re-exploration for removal of plaques
  • 18.
  • 20. External beam radiotherapy Indications Multi-focal retinoblastoma RB close to macula or optic nerve Large tumor with vitreous seeding Positioning Proper immobilization is important thermoplastic or Perspex shell with the patient supine and the chin in a neutral position Treatment is done under anesthesia: Ketamine Energy: Co-60 or 4-6 MV photons
  • 21. Technique Lateral field technique Started in 1930’s D – shaped lateral field with anterior border kept at bony orbit Disadvantage: tumor recurrence at or near ora serrata Modified lateral beam technique Two lateral opposed D-shaped fields are used
  • 22. Direct Anterior Field [Hungerford et al ] Whole eye is treated Disadvantage: Cataract formation Dose exits through brain Lacrimal gland irradiation-impaired tear production Advantages: Simple, easy to setup Reproducible Homogenously irradiates entire retina Anterior lens sparing technique (ALD) [Abramson et al ] Lateral D-shaped field: Day1-Day 4 by photons Anterior electron beam field with central circular contact lens as lens shaped shield: on Day 5
  • 23. Unilateral disease: one lateral field or 2 oblique portals [superior and inferior] Bilateral disease: parallel opposed lateral fields The anterior beam edge is placed at the bony canthus and the beam is angled 1.5 degrees posteriorly if the contralateral eye remains in place.
  • 24. Dose Ideal: 40-45 Gy, 1.8-2 Gy per fraction, 5 days a week For large tumor /vitreous seeding: 48-50 Gy Palliative radiotherapy: Extra-ocular involvement: 20-25 Gy Metastatic disease: CNS, bones: 5 Gy/1#, 15 Gy/3# Unilateral : single lateral field With anterior extension: anterior field + lateral field tilted 5-15° posteriorly Bilateral disease: parallel opposed lateral fields
  • 25. 3D CONFORMAL RADIOTHERAPY TECHNIQUE Based on 3D CT scan planning In unilateral RB, 4 non-coplanar fields are used. fields are anterior oblique: superior, inferior, medial, and lateral. 0.5-cm bolus can be used. entire retina should be treated, including 5 to 8 mm of the proximal optic nerve. Critical structures such as the opposite eye, optic chiasm, pituitary gland, brainstem, posteriormost upper teeth, and upper cervical spine. The tumor volume is treated to the 98% or 95% line,with the aforementioned organs and tissues receiving significantly less dose.
  • 26. bilateral disease, six noncoplanar fields are used: two lateral opposing, and two anterior oblique fields to each eye following the same criteria described previously. 3D CT scan reconstruction image showing beam arrangement for unilateral RB : anterior medial and lateral fields (A), anterior superior and inferior fields (B), sagittal view of composite isodose distribution (C), and axial transverse view of isodose distribution (D)
  • 27. Follow up  1st 6 months: 4-6 week intervals  Upto 3 years: at 4-6 month intervals  Later, yearly  Family history positive: All family members [other children at birth] should be examined yearly