INTRAOCULAR TUMOURS

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INTRA-OCULAR TUMOURS
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Retinoblastoma

• Definition Retinoblastoma is a proliferation of
  neural cells that have failed to evolve normally.
  Retinal cell division continues unchecked and
  results in development of retinal tumour.
  Retinoblastoma is found in infants and very young
  children.

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Epidemiology

• Disease is rare
• Frequently tumour is congenital
• Tumour is exclusively seen in infants and very
  young children
• Fellow eye is affected independently, not by
  metastasis in approximately 1/4th cases
• In approximately 10 cases a relative may have
  suffered from bilateral retinoblastoma

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Epidemiology

• Presentation usually 15 months to 24 months of
  age, most of the cases presents before the age of
  3 years
• Rare after the age of 5 years
• 30 are bilateral and 70 are unilateral
• 40 are familial and 60 are non-familial

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Epidemiology

• Genetic transmission inheritance is dominant
  with variable gene penetrance
• Related to chromosomal abnormality with deletion
  or mutation of q14 band
• Mutation of Rb1 gene has been reported

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Pathology

• Growth consists mainly of small round cells with
  large nuclei resembling the ceels of nuclear
  layer of retina. Many of the cells stain poorly
  indicating necrosis.
• Rosette shaped arrangement may be present,
  resembling rods and cones (Flexner Wintersteiner
  rosettes)
• In retinoblastoma lesions are usually multiple,
  with large lesion surrounded by multiple small
  lesions

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Pathology

• Microscopically minute deposits are seen
  scattered in various parts of globe
• Tumour may grow outwards , separating retina from
  choroid Exophytic tumour, condition resembles
  retinal detachment
• Or it grow inwards towards vitreous cavity
  Endophytic tumour seen as polypoid masses ,
  sometimes haemorrhage on the surface

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Clinical Features

• SYMPTOMS
• 1. Child is brought to ophthalmologist with
  history of yellow /white reflex in pupillary area
  sometimes called leucocoria or amaurotic cats
  eye
• 2. Squint, usually convergent , at times
  divergent
• 3. Cataract/ Bulging eye/ large eye
  (buphthalmos)

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Clinical Features

• Signs
• 1. Leucocoria
• 2. Squint
• 3. Cataract
• 4. Buphthalmos (large eye, raised tension,
  corneal edema, blue sclera)
• 5. Hypopyon / Proptosis / ocular inflammation
• 6. Mydriasis/ hyphema failed school vision test,
  dysmorphic appearance

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Stages of Retinoblastoma

• Quiescent stage (six months to one year)
• Glaucomatous stage (enlargement of globe and
  severe pain)
• Stage of extra-ocular extension (globe rupture
  usually at limbus with rapid enlargement of
  fungating growth)
• Stage of metastasis ( first in preauricular and
  neighbouring lymph nodes followed by metastasis
  to cranial and other bones)

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Metastasis

• Direct extension by continuity to the opticc
  nerve and brain
• Metastasis to other organs like liver through
  blood stream

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Reese and Ellsworth Classification

• Based on prognosis and has predictive value to
  assess likelihood to success of local treatment
• Group I Very favourable prognosis for retaining
  vision
• a. Single tumour less than 4 DD at or behind
  equator
• b. Multiple tumours of less than 4 DD size all
  at or behind the equator

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Reese and Ellsworth Classification

• 2. Group II Favourable for retaining eye sight
  
• a. single tumour 4-10 DD at or behind equator
• b. Multiple tumours 4 -10 DD in size , behind
  equator

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Reese and Ellsworth Classification

• 3. Group III possible to maintain vision
• a. any lesion anterior to equator
• b. single tumour more than 10 DD in size behind
  equator

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Reese and Ellsworth Classification

• 4. Group IV Unfavourable for maintenance to eye
  sight
• a. Multiple tumours some larger than 10 DD in
  size
• b. Any lesion extending anterior to ora serrata
• 5. Group V Highly unfavourable for maintaining
  eye sight
• Massive tumours involving more than half retina
  and vitreous seeding

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Diagnosis

• Clinical presentation
• USG B Scan
• X- ray orbit, skull etc
• CT Scan
• Ant chamber fluid cytotology
• Biopsy (in cases of extra-ocular extension)
• Raise Lactic dehydrogenase activity in aqueou
  relative to the serum
• MRI for estimation of degree of differentiation
  

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Treatment

• Prognosis of retinoblastoma if left untreated ,
  is always bad
• Prognosis is fair if extra-ocular extension is
  avoided

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Differential Diagnosis

• Pesudoglioma
• 1. Inflammatory deposits in the vitreous
• 2. Toxocariasis
• 3. Congenital defects (PHPV and Norrie disease,
  Colobomas of choroid and disc)
• 4. Retrolental fibroplasis
• 5. Cataract
• 6. Retinal detachment, Retinal dysplasia,
  tumours other than Retinoblastoma, coats
  disease, Vit. Haemorrhage , uveitis

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Treatment

• Small tumour
• Local modalities like cryotherapy (for ant
  lesion), Photocoagulation for posterior lesion,
  brachytherapy with Co 60 or 125 I and
  steriotactic radiation
• Suturing of radioactive cobalt discs it
  deliver a dose of 4000 rad to summit of the
  tumour in one week.

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Treatment

• Photocoagulation
• Placing a double row of confluent burns around
  each tumours with a photocoagulator.
• Repeat treatment may be required.
• Cryotherapy- For anteriorly located tumours.
  Under direct visualization freezing until ice
  ball incorporates entire tumour. Tumour is
  allowed to thaw and refreez- thaw cycle is
  repeated 3-4 times.

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Treatment

• 2. Enucleation
• 3. Exenteration of the orbit
• 4. External beam radiation
• 5. Chemoreduction with chemotherapy (Vincristine,
  etoposide and carboplatin)

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Malignant Melanoma

• Malignant Melanoma is highly malignant tumour
  arising from the outer layers of choroid. It is
  commonest intra-ocular tumour.

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Clinical Features

• Adults between the age of 40 60 years affected.
• Less common amongst African and Asians

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Symptoms

• Visual acuity is markedly affected when tumour is
  located centrally near macula
• In glaucomatous stage patient presents with
  severe pain in and around eye
• In stage of extra-ocular extension growth in
  orbit / fungating mass
• In stage of metastasis varied presentation
  depending on organ involved

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Signs

• Tumour is primarily single and unilateral
• A lens shaped mass raising the Retina above
• It stretches the Bruch membrane which ruptures
  and then tumour proliferates through the opening
  and retinal pigment epithelium to form a globular
  mass in the subretinal space
• Lens becomes opaque as its nutrition suffers

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Signs

• Tumour fills the globe then perforate the sclera
• Orbital extension may occur in early stage due to
  spread along vortex vein or ciliary vessels
• Orbital tissue is infiltrated with tumour cells
  presenting as proptosis
• Lymph nodes are not commonly affected

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Signs

• Distant metastasis occurs to liver and elsewhere
• Growth is usually pigmented but may be
  occasionally unpigmented ( pigments are chiefly
  melanin)
• Surface of tumour may have mottled orange and
  black appearance

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Signs

• Flat malignant melanoma
• Choroid is widely infiltrated so that a uniform
  thickening results with shallow Retinal
  Detachment

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Clinical stages

• The quiescent stage
• The Glaucomatous stage
• The stage of extra-ocular extension
• The stage of metastasis

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Pathology

• Composed of spindle shaped cells
• Cells may also be cylindrical or palisade-like,
  arranged in columns or around blood vessels
• Most of the tumours are of mixed type
• Spindle A
• Spindle B
• Epitheloid (most malignant)
• Mixed
• Contains variable amount of reticulin fibres

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Differential Diagnosis

• Choroidal naevus
• Cavernous haemangioma
• Posterior scleritis

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Diagnosis

• Ultrasonography A and B scan
• Radioactive tracers- increased rate of phosphate
  uptake and its retaintion for longer time
• Fluorescein Angiography- double circulation with
  increased fluorescence in the mass (Indocyanine
  green angiography)

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Treatment

• A pigmented lesion with diameter larger than 5 DD
  (7.5 mm) should be considered a malignant
  melanoma until proved otherwise
• Goals eradication of tumour , maintenance of
  vision and cosmetically acceptable eye
• Tumour less than 10 mm and upto 2 mm thickeness
  is treated by brachytherapy using
• Radioactive discs of gold, cobalt 60 or iodine
  125

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Treatment

• For treatment of small tumour
• External beam radiation
• Cryotherapy
• Laser ablation
• Transpupillary thermotherapy
• For treatment of medium size tumour (10-15 mm in
  diameter and 3-5 mm in height)
• Plaque or external proton beam radiation

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Treatment

• Enucleation
• Exenteration
• PROGNOSIS
• Disease is usually fatal in 5 years if not
  treated successfully
• In cases with metastasis death usually occurs
  within a year of detection of metastasis