RETINOBLASTOMA OR GLOMA OF RETINA:

Malignant proliferation of neurologia such as occur in the brain and optic nerve are rare in the retina.

The usual maliganant growth of the retina is due to proliferation of neural cells (inner or outer nuclear layers) and is better termed retinoblastoma

1)Age incidence-it is probably always congenital and occurs in childern under 5 years of age.

2)Usually it starts in one eye. When bilateral the second eye is affected independently and not by metastasis or continuity via the optic chiasma.

3)Heredity: Most of the cases are sporadic but a familial from is seen in 4,5% cases.

The tumour consists of small round cell with large basophilic nuclei .The cells may be arranged in

rosettes, pseudorosettes and fleurettes .

a)Rosettes: The cells tend to be arranged around acentral space.

Rosettes are of two types: 1)Flexner-Wintersteiner which are typical of retinoblastoma and contain mucopolysaccharide in the lumen

2)Homer-Wright which may be found in other tumours also and contain fibrillar material in the lumen

b) Pseudorosettes: Here, the cells tend to be arranged around blood vessel.

c)Fleurettes :The arrangement of retinoblastoma cells here resemble the pattern of a flower

The cells show a tendency to necrosis and the mass is permeated by calcium deposits. The tumour may show multiple foci which are independent

and do not disseminate from a primary focus.

The cells may be poorly differentiated or well differentiated (neuro-epithelioma) .

The mass may grow

1(Outward separtating retina from choroid (glioma exophytum ).

2)Inward-toward vitreous (glioma (endophytum

3)Sideward (glioma planum).

Mother bring the child with complaints of:

1 )Amaurotic (blind) cats eye reflex (yellow reflex) or leucocoria (white reflex).in the pupillary area.

2)Visual disability :the child bumps into things on the side of the affected eye indicating visual loss.

3)Convergent squint due to poor vision from involvement of macular area.

4 )Red painfuleye.

These can be divided into 4 stages.

1)Quiescent stage:

Externally the eye is quiet .With ophthalmoscope small yellow or whitish plaques are seen in one or more location in the retina .The plaques increase in size and sometimes fuse into a single large yellow or whitish mass, causing a yellow or white reflex in the pupillary area (amaurotic cats eye or leucocoria ).The surface of the growth shows new vessels, spots of haemorrhage and even calcification. The pupil usually becomes dilated.

2 )Stage of glaucoma:

The causes of secondary glaucoma are: a) Involvement of vortex veins.

b) Involvement of the angle of AC by extension of growth.

c) Iris lens diaphragm pushed forward by growth giving rise to peripheral anterior synechiae.

Owing to the extensibility of the sclera (upto the age of 3 years) under the increase of pressure, the entire cornea and sclera stretch so the globe enlarges and presents the picture of secondary buphthalmos . In addition acute inflammatory reaction (uveitis) results from extensive necrosis within the tumour.

a)Circumcorneal congestion and later conjunctival congestion and chemosis

b)cornea large and hazy. c)AC may be cloudy and may show pseudohypopyon (deposit of malignant cells unlike inflammatory cells as in true hypopyon)

d)Vitreous :is frequently filled with tumour producing an appearance resembling vitreous abscess.

Pain is sever, but is relieved when the tumour bursts through the sclera usually

at the limbus.

3)Stage of extraocular extension:Extraocular extenstion occurs through the site of perforation of:

a) Anterior and posterior ciliary arteries.b)Vortex veins.c) A long the optic nerve.d) Tumour bursts through the sclera usually at

the limbus.

The eye ball show proptosis and restriction of movements.

The extraocular portion of the tumour grows rapidly and produces a fungating mass

protruding between the lids.

4 )Stage of metastasis

a)Direct extension by continuity along the optic nerve and brain.

b)Preauricular and cervical lymphnodes.

c)Cranial and other bones like humerus ,sternum.d)Liver is rarely affected.

Retinoblastoma must be distinguished from a number of condition which have been grouped together under the term pseudoglioma:

1)Cyclitic membrane

2)Suppyrative endophthalmitis 3)T.B. of the choroid –confluent or

conglomerate type

4)Toxocara infestation (visceral larva migrans)

Congenital Malformation

1)Persistent hyper-plastic primary vitreous.

2)Congenital detached retina.

Retrolental fibroplasia

Intraocular haemorrhage(1)

Intraocular haemorrhage(2)

Retinoblastoma

Pseudoglima

1)High or normal intraocular pressure

Low intraocular pressure

2)Eyeball may be normal size or enlarged. (buphthalmos) as evidenced by the size of the cornea.

Usually micophthalmos as evidenced by size of corenea

Retinoblastoma(1)

Retinoblastoma(2)

Pseudoglioma

Pseudorosettes

3)New bright red vessels with haphazard branching may be seen on white or yellow mass in pupillary area.

Dark retinal vessels with dichotomous branching may be seen on white or yellow mass in pupillary area.

4)X-ray shows a)calcification (mottled

appearance) b)enlarged optic foramen

No calcification Normal optic foramen

5)Progressive Usually non progessive

In all the cases 1% atropine should be instilled and both eyes examined thoroughly with the opthalmoscope under general anaesthsia. Tension should be checked with the tonometer.

In some cases it is impossible to be certain of the diagnosis.

Considering that the life of the patient is stake and that the eye in any case is useless as an organ of sight ,these cases should be treated as retinoblastoma.

1)Quiescent and glaucomatous stage:Enucleation at the earliest opportunity is the safest and still the most effective method of treatment of retinoblastoma .In this operation the importance of excising as long a portion of the optic nerve as possible cannot be over stressed .This is achieved by pulling on 2 traction sutures passed through the insertion of the 2 horizontal rectus muscles and then passing the enucleation scissors along the nasal wall of the orbit towards the optic foramen.

2)Stage of extraocular extension :Exenteration of the orbit has to be done i.e. removal of all the orbital contents including the periosteum

3) Stage of distant metastasis

a)Radiotherapy with deep X-ray to the orbit

b)Chemotherapy

i)T.E.M(Triethylene melanine) 0.08mg/kg body weight I.M.

ii)Cyclophosphamide- given as oral tablet or I.V.

Cryotherapy or photocoagulation is indicated in cases where one eye has been enucleated and small tumour plaques are seen in the seen in the seeing eye. Cryotherapy is employed in peripheral lesions with hazy media . photocoagulation is used in

posterior lesion with clear media.

is poor .Death usually occurs due to intracranial extension.

2)MALIGANT MELANOMA OF CHOROID:

Malignant Melanoma(1)

Malignant Melanoma(2)

1)It may be mesodermal or neurectodermal in origin.

2)Age incidence :It usually occurs in adults between 40-60 years.

3)It is always primary ,single and unilateral.

The tumour consists of spindle shaped or cylinderical cells, arranged in columns or around blood vessels ;most tumours are mixed celled .Silver staining reveals a variable amount of reticulin fibres . The growth is usually pigmented (melanin).It starts in the outer layers of choroid .It forms first an almond shaped mass, raising the Bruchs membrane ;this membrane later ruptures .The tumour then proliferates to form a globular mass in the subretinal space .It thus forms a dumb –bell shaped mass. The retina is detached ,the subretinal space being filled with

exudative fluid. .

The earliest patients to seek advice are those in whom the tumour is near the macula.

It causes:1) Diminished vision

2) Micropsia or macropsia. These are due to separation or crowding of retinal elements respectively.

3) metamorphopsia : distortion of an object. 4) photopsia – flashes of light .

In cases where the tumour is in the periphery the patient may

present with a red painful eye (due to glaucoma ).

These can be divided into 4 stages like retinoblastoma

1) Quiescent stage .2) Stage of glaucoma – the causes of secondary

glaucoma are

-involvement of vortex vein

-iris lens diaphragm pushed forwared by growth giving rise to peripheral anterior synechiae . In addition acute inflammatory reaction (uveitis)

results from extensive necrosis within the tumour.

a) Ciliary and conjunctival congestion b) Cornea hazy

c) fundus shows secondary retinal detachment . pain is severe.

3) Stage of extraocular extension : Extraocular extension occurs

through the site of perforation of : a) Anterior and posterior ciliary arteries b) Vortex veins c) Tumour bursts through the sclera

The eyeball shows proptosis and restriction of movements. The extraocular portion of the tumour grows rapidly and produces a black , fungating mass between the lids.

4) Stage of distant metastasis : liver is affected. Enlarged liver and leucocoria (or artificial eye if the eye has been enucleated ) in an adult think of malignant melanoma of choroid.

Primary retinaldetachment

Secondary retinal detachment due to malignant melanoma of choroid

1)furrowe & ridges seen on retinal detachment

Rounded solid lookingmass.

2) No pigmentation No new blood vessels on retinal datachment.

Pigmentation in the centre and new blood vessels present on retinal

detachment.

Primary retinal detachment

3)Tension normal or low

Tension high

4) Trans illumination negative.

positive

1)Quiescent and glaucomatous stage: Enucleation of eyeball.

2) Stage of extraocular extension – exenteration of the orbit has to be done i.e. removal of all the contents of the orbit including the periosteum .

3) Stage of distant metastasis – radiotheraby with deep X- Rays to the orbit.When the tumour is small and the affected eye is the only seeing eye , destruction by photocoagulation or suturing cobalt disc to the sclera over the site of the tumour is indicated.Is poor. Death usually occurs after some years.

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