Intraocular tumors

25 ago, if we found a choroideal melanoma

in an eye, the eye had to be enucleated.

Pigmented tumor in an eye

(melanoma chorioideae)

An eye surgically removed because of

choroideal melanoma, the eye is cut in half

Since 1986 intraocular tumors are treated with Ruthenium applicators at the Department of

Ophthalmology, University of Debrecen.

Ophthalmic applicators used for

the radiotherapy of intraocular tumors

Applicator on the eye,

isodose curves

Due to early diagnosis, and to effective radiotherapy the radical surgeries used before the irradiation era

are very rarely used.

Removal of the eyeball

(enucleatio bulbi)

Orbital plasty

(exenteratio orbitae)

The outer surface of the applicators is covered by a

radioprotective layer, which is missing on the inner surface.

Radiotransmitting area on the inner surface

Different applicators are used for the irradiation of tumors with different size

and different localization.

The applicators are sutured on the surface of the eye,

and removed after sufficient irradiaton dose is achieved.

Implantation of anterior

segment applicator Implantation of posterior

segment applicator

Planning of the irradiation

A prominent, half spere shaped intraocular tumor,

turns into a flat scar following irradiation.

Melanoma before irradation The same tumor 1 year

after the irrradiation

Malignant melanomas can be differentiated from benign nevi

with fluorescein-angiography with very high precision.

Color fundusphoto Angiographic appearence

of the same fundus

With ultrasonography the size (prominence) and the inner

structure (reflectivity) of intraocular tumors are examined.

Different tumors have different ultrasonographic appearence.

By transpupillar and by transscleral illumination of the

eyeball (diaphanoscopy) the localization of the tumors

in the eye can be determined.

Strate and bent fiberoptic light sources

Transpupillar diaphanoscopy Transscleral diaphanoscopy

K.J., a 72 years old female patient, who formerly lost her left

eye in an accident, received irradiation with an applicator on

the other eye because of choroideal melanoma in the year of

1986. She was the first patient we treated with this method.

Right after irradiation Three months later

After irradiation the melanoma gradually turns into a flat scar in

6 to 12 months, during an after this period the patients have to

be regularly controlled for five years.

A melanoma right after irradiation Three months following irradiation

In half a year most of

the tumor cicatrized

Flat pigmented scar developed

in the place of the former tumor

Besides choroideal melanoma seen

in adults, the second most frequent

malignant intraocular tumor is retino-

blastoma, that we see in newborns or

in small children.

Based on clinical appearence four types of retinoblastoma are known:

Intraretinal Endophytic

Exophytic Peripherial

Different size retinoblastomas are treated differently

Small retinoblastomas Medium size retinoblastomas

Big retinoblastomas Those that fill up the eye,

and cause retina detachment

Unilateral retinoblastoma

Unilateral retinoblastomas are usually

diagnosed in older children (not new-

borns), in advanced stage when enuc-

leation is the only possible therapy. In

cases with small tumors local therapy

is also possible, but this occurs very ra-

rely. Unilateral retinoblastomas are not

treated with systemic cytostatic drugs!

Bilateral retinoblastomas are usually of different

severity in the two eyes when diagnosis is made.

One eye of the child usually has to be enucleated due to a retinoblastoma filling the eye.

Small or medium size herds in the other eye usually can be treated locally.

In the last 15 years local treatment was almost always Ruthenium

applicator treatment (in some cases transscleral cryoapplication).

One month after irradiation Three months after irradiation

Six months after irradiation More than one herd can be

treated with one applicator

Transpupillary thermotherapy (TTT) performed with a diode

laser started a new era in the treatment of intraocular tumors.

The benefits of transpupillary thermotherapy

• Small tumors can be treated with TTT alone.

• TTT can be a good additive treatment to irradiation in case

of medium size tumors.

• In some big tumor cases with sandwich therapy enuclea-

tion can be avoided.

• TTT is more selective, causes less damage in the eye than

radiotherapy .

Children with bilateral retinoblastomas get

additional cytostatic treatment, most of

them at the Department of Pediatric

Hemato-Oncology, Institute of Pediatrics,

University of Debrecen. Children coming

from other parts of the country at other

Pediatric Oncological Centers in Hungary.

The CT scans of one of our first bilateral retinoblastoma

patients, who is presently 12 years old, and goes to normal

school

Before treatment: in the right eye a big,

in the left egye a small retinoblastoma,

with juxtapapillary localization.

Four years following the enucleation

of the right eye, and the irradiation of

the left eye. Note the normal appear-

ance of the left eye, without relapse.

Molecular genetics of retinoblastoma

• Retinoblastoma (Rb) gene is in

the 13q14 chromosomal re-gion.

• The Rb is a tumor suppressor gene, that palys a role in the regulation of the mitotic cell cycle.

• Retinoblastoma develops if the two allels of the retinoblastoma gene are lost or suffer mutation (Knudson’s ”two hits hypothe-sis”)

13q14

Genetic counselling in retinoblastoma

Retinal angioma treated with Ruthenium applicator

(angiomatosis retinae, von Hippel-Lindau disease)

Retinal angioma with feeding

vessels right after irradiation

Cicatrized angioma, closed feeding

vessels, half year following irradiation

By irradiation of metastatic tumors in the eye the quality of

life of patients with multiple metastais can be improved.

Intraocular metastasis of prostata

carcinoma right after irradiation Flat scar in the level of the sclera

one month following irradiation

We published our first 15 years’ results in 2005 in the journal

„Magyar Onkológia”, on patients whose follow up period was at

least 5 years at that time.

The distribution of intraocular tumors, according

to diagnosis, treated with Ruthenium applicators

at our Department between 1986 and 1999

On this table those patients are shown who were treated in the fol-lowing 5 years, and whose follow up period in at least 5 years now. In this period small tumors were already treated with diode laser.

The distribution of intraocular tumors, according

to diagnosis, treated with Ruthenium applicators

at our Department between 2000 and 2005