TREATMENT
The treatment of retinoblastoma is determined individually for each child, as the initial situation varies greatly from patient to patient.
Therapy-
PLANNING
Once the diagnosis has been established, treatment planning takes place.
In order to be able to provide treatment that is tailored to the individual patient (risk-adapted), the treatment team takes into account certain factors that influence the patient’s prognosis (so-called risk or prognosis factors) when planning.
The spread of the retinoblastoma at the time of diagnosis is an important prognostic factor and therefore a key criterion when choosing the most suitable therapy. This also takes into account whether the disease is unilateral or bilateral.
The extent of the disease gives doctors an indication of how the patient will respond to a particular treatment method, whether vision can still be expected in one or both eyes after the therapy and how high the risk of further tumor growth or a relapse of the disease will be.
The patient’s age and state of health and knowledge of a genetic predisposition to the disease are also taken into account when planning treatment.
Stages of spread of retinoblastoma
Depending on its extent, retinoblastoma is divided into different stages (so-called classification).
The first decisive factor is whether it is an intraocular retinoblastoma, i.e. only the eye/eyes are affected, or whether the disease has also spread to tissue/organs outside the eye/eyes or to other parts of the body (extraocular retinoblastoma).
The extent of the tumor is always assessed separately for both eyes.
There are several classification systems for the staging of intraocular and extraocular retinoblastoma.
The most common classification of intraocular disease is the “International Classification of Retinoblastoma (ICRB, Philadelphia version)”. It takes into account the size and location of the tumor as well as the presence and extent of vitreous seeding and distinguishes five disease stages (A to E) accordingly.
In the “International Retinoblastoma Staging System (IRSS)”, extraocular retinoblastoma is divided into four stages according to tumor removal (enucleation) (IRSS I-IV).
A retinoblastoma that is not enucleated is referred to as stage 0.
Another classification system – the TNM classification for retinoblastomas – combines information on intra- and extraocular disease.
THERAPY
The choice of the appropriate form(s) of therapy depends primarily on the size and location of the retinoblastoma.
The treatment options for retinoblastoma include surgery, chemotherapy (systemic or local), radiotherapy (brachytherapy or percutaneous radiotherapy), laser therapy, cryotherapy and thermotherapy. In the case of advanced disease, high-dose chemotherapy followed by autologous stem cell transplantation may also be an option in some situations.
Which procedures are used depends primarily on whether one or both eyes are affected by the tumor, how advanced the disease is at the time of diagnosis (intraocular or extraocular retinoblastoma and the respective stage) and whether one or both eyes can still be expected to have vision after treatment.
The age of the child is also taken into account when planning treatment. The aim of any therapy is to completely destroy or remove the tumor and thus cure the cancer.
The preservation of life always takes precedence over the preservation of sight.
In principle, two
treatment strategies are possible
1. eye-preserving therapy using laser, cryotherapy, thermotherapy, radiotherapy and/or chemotherapy
Eye-preserving treatment is preferred whenever possible. In the presence of single, small retinoblastomas, the treatment of choice is local ophthalmologic therapy (focal therapy).
The choice of the appropriate form(s) of therapy depends primarily on the size and location of the retinoblastoma:
Laser therapy
Laser therapy is mainly used for small tumors (less than 2 mm in height); a laser beam is directed at the tumor through the pupil under general anesthesia.
The tumor is destroyed by the heat of the laser beam.
Cryotherapy
Cryotherapy uses particularly low temperatures.
The tumor is located from the outside with the help of an ophthalmoscope and a metal probe and frozen several times.
This destroys the cold-sensitive tumor cells.
This form of therapy is suitable for tumors (up to around 3-4 mm high) that are located in the anterior section of the retina (peripheral).
It can also be used to treat localized vitreous seeding.
Thermotherapy
Thermotherapy (or thermochemotherapy) is a form of laser therapy that is combined with systemically administered chemotherapy.
It is mainly used for tumors at the posterior pole of the eye.
Brachytherapy
Brachytherapy (short-distance irradiation) is used to irradiate individual retinoblastomas (between 4-5 mm in height) in easily accessible areas.
For this purpose, a radioactive radiation carrier (e.g. a ruthenium applicator) is surgically placed on the sclera in the area of the tumor and left there (usually for several days) until the desired radiation dose has been administered.
The radiation is only delivered in the direction of the tumor.
This allows a high dose of radiation to be delivered directly to the tumor, while the adjacent tissue is largely spared.
The radiosensitive retinoblastoma is gradually transformed into inactive scar tissue over weeks, months or even years.
Chemotherapy
If the tumors are already too large for such treatment, chemotherapy can be carried out in some cases with the aim of reducing the size of the tumor (chemoreduction) in order to subsequently enable local treatment. As a rule, several cytostatic drugs are used at the same time (polychemotherapy).
The drugs are administered intravenously.
They are distributed throughout the body via the bloodstream and thus also reach the blood vessels of the tumor in order to take effect there.
In order to achieve high chemotherapy concentrations directly in the eye and at the same time avoid or reduce the side effects of systemic chemotherapy, local chemotherapy procedures have been used for some time as part of eye-sustaining treatment.
Good to know
The aim of eye-preserving therapy is to inactivate the tumor and at the same time preserve vision without risking the patient’s life.
All of these forms of therapy can be used alone or in combination with other therapies.
2. surgical removal of the tumor by removing the eye (enucleation) and, if necessary, supplementary (adjuvant) forms of therapy to consolidate the success of the treatment
If the disease is already advanced, removal of the eye, known as enucleation, is usually unavoidable. This is often the case with unilateral retinoblastomas, which are usually already larger at the time of diagnosis.
In the case of bilateral retinoblastomas, the tumors have usually grown differently so that the two eyes have different stages of disease.
In these cases, the more severely affected eye is usually surgically removed, unless both eyes can be preserved.
To remove the tumor, the entire eyeball must always be removed along with as much of the optic nerve as possible.
Patients who show certain histological risk factors (infiltration of the choroid, sclera or optic nerve) after enucleation and thus tumor spread outside the eye must receive additional (adjuvant) treatment in order to reduce the risk of further spread of the tumor via the bloodstream to other parts of the body and/or via the optic nerve to the brain.
Even if metastases are already present, further treatment is required. The more advanced the disease, the more intensive and complex the therapy will be.
Treatment options include: systemic chemotherapy alone, combined chemotherapy and radiotherapy (the latter limited locally to the eye socket) and multimodal therapy consisting of chemotherapy, high-dose chemotherapy with autologous stem cell transplantation and radiotherapy.
PROGNOSIS
Over 95% of children with retinoblastoma in Germany can now be cured in the long term thanks to modern diagnosis and treatment methods.
Children with a unilateral retinoblastoma have a healthy eye with no visual impairment and can lead a completely normal life. The majority of children with bilateral retinoblastoma also retain at least one eye with residual visual acuity.
The prognosis for the individual patient depends in particular on how advanced the disease is at the time of diagnosis (stage of disease) and whether the retinoblastoma is hereditary or non-hereditary.
Retinoblastomas that are confined to the eye – or eyes – at the time of diagnosis (intraocular retinoblastoma) can be treated better than diseases that are more advanced in their growth; they are therefore generally associated with a more favorable prognosis.
Patients with a hereditary retinoblastoma have a less favorable overall prognosis than patients with the non-hereditary form. This is due to the fact that with hereditary disease, regardless of treatment, there is a genetically increased risk of developing a second malignant tumor (for example a soft tissue tumor or osteosarcoma). This risk increases further if the eyeball is irradiated as part of the treatment. Around 5% of children with a hereditary, initially unilateral retinoblastoma develop a retinoblastoma on the opposite side within a year and a half of the initial disease.
Sources: Maria Yiallouros, editorial team kinderkrebsinfo.de
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Berlin | Westend
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Clinical Department of Pediatric Hematology/Oncology
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Auenbruggerplatz 4
A-8036 Graz
T +43-316-385-3817
gerald.langmann@meduni-graz.at
AUSTRIA
Werner Wackernagel, MD
Department of Ophthalmology Medical University Graz
Auenbruggerplatz 4
8036 Graz Austria, EU
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BELGIUM
Cliniques Universitaires St-Luc
Avenue Hippocrate, 10
Brussels, B 1200
Professor Patrick de Potter
T +322 02 764 19 52
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Department d’Ophtalmologie – Institut Curie
26, rue d’Ulm,
Paris, F-75231
Laurence Desjardins, MD
T +33 1 4432 400
ITALY
Instituto di Scienze Oftamologiche
Universita Degli Studi di Siena
Strad Delle Scotte
53100 Siena, Italy
Teodora Hadjistilianou, MD
T +39-577-48070
SWEDEN
St. Erik’s Eye Hospital
Fleminggatan 22
S-11282 Stockholm, Sweden
Prof. Erik Kock, MD
F +46 8 672 3375
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Francis Munier
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