Retinoblastoma Treatment in Turkey: A Guide for Parents

Retinoblastoma is a cancer that starts in the retina — the thin, light-sensitive layer at the back of the eye — and it affects babies and young children. It is curable in the great majority of children when it is found while still inside the eye, and modern care aims to save the child's life first and then, whenever it is safe, the eye and useful vision. This guide explains the disease step by step for parents, including what retinoblastoma treatment in Turkey involves for families who travel to Antalya from abroad.

Many families first become worried when a white glow appears in one pupil in a photograph — the most common first sign of the disease.

What is retinoblastoma?

The retina develops rapidly before birth and in the first years of life. Retinoblastoma begins when an immature retinal cell loses both working copies of a gene called RB1, which normally acts as a brake on cell division. Without that brake the cell keeps dividing and forms a tumor. In a small number of children the tumor is driven instead by extra copies of another gene, MYCN.

A child can have one tumor or several, in one eye (unilateral) or both eyes (bilateral). Tumors can also shed small clusters of cells, called seeds, into the clear gel that fills the eye (the vitreous) or under the retina; seeds are harder for treatment to reach. Without treatment, retinoblastoma can grow along the optic nerve toward the brain or spread through the blood to the bones and bone marrow, which is why it is always treated promptly. With timely treatment, the great majority of children survive.

Who gets retinoblastoma, and how common is it?

Retinoblastoma is rare: it occurs in about 1 in 15,000–20,000 live births, or roughly 8,000–9,000 children worldwide each year. It accounts for about 3% of cancers in children under 15 in the United States and affects boys and girls of all ethnic backgrounds at similar rates.

About two-thirds of children are diagnosed before their second birthday and almost all before age 5. About 1 in 3 has tumors in both eyes; these children tend to be diagnosed earlier — on average at around 15 months, compared with about 24 months when one eye is affected.

Heritable and non-heritable retinoblastoma

• In heritable retinoblastoma, the first RB1 change is present in every cell of the body (a germline variant). The American Cancer Society puts this at about 4 in 10 children; other sources give 25–40%. It includes every child with bilateral disease and about 15% of children with one eye affected — and about 9 in 10 of these children are the first in their family to have it.
• In non-heritable retinoblastoma, both changes occur only in the tumor cells of one eye.

The form shapes the screening of brothers and sisters, the choice of treatment (radiation is avoided wherever possible in the heritable form) and lifelong health checks. Our page on retinoblastoma genetics and family screening covers RB1 testing, risks for relatives and screening schedules.

What are the symptoms and signs of retinoblastoma?

Babies do not complain that they cannot see, and a child with one healthy eye can behave normally, so retinoblastoma is usually noticed by parents, relatives or a doctor:

• A white pupil (leukocoria) — one pupil looks white or yellowish in flash photos instead of red, sometimes called a cat's-eye reflex. This is the most common first sign.
• A squint (strabismus) — an eye that turns in or out, often because a tumor sits at the center of the retina.
• A red, painful or swollen eye without obvious cause, an enlarged eye, a change in iris color or, in advanced disease, a bulging eye.
• Poor vision, noticed mainly in older children.

In children younger than 5, a white pupil is the first sign in about 60% and a squint in about 20%, with signs of inflammation in about 5%. In older children, a white pupil and reduced vision each account for about 35% of cases.

A white pupil can also be caused by Coats disease, persistent fetal vasculature, toxocariasis, cataract, retinal detachment or a harmless reflection from the optic nerve — see our guide to a white pupil in photos. The American Academy of Pediatrics recommends checking the red reflex before a newborn leaves the hospital and at every well-child visit.

How is retinoblastoma diagnosed?

The diagnosis is made by an ocular oncologist — a retinoblastoma specialist — from the tumor's appearance and from imaging. Unlike most cancers, retinoblastoma is not biopsied, because a needle could release cancer cells outside the eye. Instead, several tests build a precise map of every tumor:

• Examination under anesthesia (EUA): while the child sleeps under a short general anesthetic, both retinas are examined out to the far edges. EUAs are repeated throughout treatment to measure the response.
• Wide-field photography (for example with a RetCam camera) records each tumor and any seeds for exact comparison over time.
• Ultrasound (B-scan) measures the tumor and shows calcium deposits inside it as bright echoes with a shadow — a finding regarded as essentially diagnostic.
• Optical coherence tomography (OCT) gives cross-sectional images of the retina. Hand-held OCT, used during the EUA, can reveal tiny tumors not yet visible and helps separate tumor from harmless look-alike lesions.
• MRI of the brain and orbits checks the optic nerve and the pineal gland. CT is avoided because its radiation adds to the long-term cancer risk of children with the heritable form.

Every child should be offered RB1 genetic testing, usually from a blood sample. Bone marrow or spinal fluid tests are generally reserved for high-risk features or signs of spread, and testing tumor DNA in fluid from the front of the eye (a "liquid biopsy") is still being studied. OCT in retinoblastoma has been part of Prof. Türkoğlu's own published work (see below); for imaging across all eye tumors, see how eye tumors are diagnosed.

How is retinoblastoma classified?

Two systems are used side by side. The group (A to E) describes the tumor inside the eye and predicts how likely it is that the eye can be saved; the stage describes spread beyond the eye and whether the child has the heritable form.

Groups A to E

There are two closely related versions of the grouping — the International Intraocular Retinoblastoma Classification (IIRC, from Los Angeles) and the International Classification of Retinoblastoma (ICRB, from Philadelphia). They use the same letters but word some groups differently, so check which version a report uses. The table follows the Philadelphia wording.

AJCC stage and the "H" category

The eighth edition of the American Joint Committee on Cancer (AJCC) system uses the TNM letters plus one more. cT1–cT3 describe tumors confined to the eye; cT4 means tumor outside the eye, along the optic nerve or in the orbit; N and M record spread to lymph nodes and distant sites. H records heritability: H1 for bilateral or trilateral disease, a family history or a germline RB1 variant; H0 when high-sensitivity testing finds normal RB1; HX when unknown — the first cancer staging system to include a heritable trait. In the Global Retinoblastoma Outcome Study, children with cT4 disease had about 9 times the risk of death of children with cT1 tumors. See also how eye cancers are staged.

What are the goals of treatment?

Retinoblastoma treatment follows a clear order: first save the child's life, then the eye, then as much vision as possible. A plan that keeps an eye but leaves a real risk of spread is not acceptable — which is why removing an eye is sometimes the safest choice even when other options sound gentler.

Care is organized by a multidisciplinary team: the ocular oncologist, pediatric oncologists, interventional neuroradiologists, pediatric anesthesiologists, ophthalmic pathologists, geneticists and ocularists, who make artificial eyes, with radiation oncologists when needed. Prof. Türkoğlu evaluates your child's case, explains the options and plans treatment with you. Where a method needs specialized facilities — such as intra-arterial chemotherapy, systemic chemotherapy or radiotherapy — it is delivered together with the relevant specialist teams.

Which treatments are used for retinoblastoma today?

Most children receive a combination of treatments, chosen for each eye separately.

• Intra-arterial chemotherapy (IAC). A very thin catheter is guided from the groin to the ophthalmic artery, which supplies the eye, and chemotherapy (usually melphalan) is infused directly into it. Developed from work at the National Cancer Center in Tokyo in the late 1980s, it is now a main treatment for advanced disease in one eye, performed under general anesthesia by an interventional neuroradiology team.
• Intravitreal chemotherapy. A tiny dose of melphalan or topotecan is injected into the vitreous to treat seeds, with the entry site frozen as the needle is withdrawn to stop tumor cells escaping. Intracameral chemotherapy, for seeds in the front chamber of the eye, is an emerging option reported in small case series.
• Systemic chemotherapy (chemoreduction). Vincristine, etoposide and carboplatin through a vein, for up to 6 cycles, shrink tumors in both eyes at once. Pediatric oncologists give it for bilateral disease, very young babies, high-risk pathology after enucleation and spread outside the eye.
• Focal consolidation. Laser photocoagulation and transpupillary thermotherapy (a gentle heat laser) treat small tumors at the back of the eye; cryotherapy freezes small tumors toward the front.
• Plaque brachytherapy. A small radioactive disc stitched temporarily to the eye wall treats a single medium-sized tumor or a recurrence; see plaque brachytherapy.
• External beam radiotherapy. Now avoided whenever possible: in the heritable form it more than triples the risk of later cancers, especially in the first year of life, and can affect the growth of the bones around the eye.
• Enucleation (removal of the eye). For very advanced eyes (usually Group E), removal is often the safest cure, particularly when the other eye is healthy. The eye is removed whole with about 10 mm of optic nerve and an implant is placed. If the pathology shows high-risk features — such as tumor in the optic nerve behind the eye wall or massive invasion of the choroid — about 6 months of chemotherapy lowers the risk of spread. See enucleation and the artificial eye.

Each form of chemotherapy is explained on our page on intra-arterial and intravitreal chemotherapy.

What is the retinoblastoma survival rate, and can the eye be saved?

Survival depends above all on how early the cancer is found. According to the American Cancer Society, more than 9 in 10 children with retinoblastoma in the United States are cured; when the tumor is still inside the eye, 5-year survival is above 95%. With spread to the orbit or nearby lymph nodes it falls to about 60–90%, and it is below 20% once the cancer reaches the brain or spinal fluid.

The Global Retinoblastoma Outcome Study followed 4,064 children from 149 countries diagnosed in 2017:

The gap reflects mainly late diagnosis and limited access to specialized care: the cancer had already spread outside the eye in 42.9% of children in low-income countries, compared with 0.8% in high-income countries.

Saving the eye

• At Wills Eye Hospital in Philadelphia, 74% of 341 eyes treated with intra-arterial chemotherapy were saved at 5 years; as the first treatment, 100% of Group B and C eyes, 86% of Group D and 55% of Group E eyes, with no metastasis or death in the series.
• In the long-term Tokyo series (408 eyes, 1988–2007), 5-year eye salvage ranged from 100% in Group A to 30% in Group E; many eyes also received radiotherapy.
• A Cochrane systematic review published in 2026, pooling six studies, concluded that for advanced retinoblastoma in one eye, intra-arterial chemotherapy probably roughly doubles the chance of keeping the eye without progression at 2 years compared with intravenous chemotherapy (risk ratio 1.97), with similar overall survival and possibly fewer side effects elsewhere in the body.
• Without external radiotherapy, about 78% of eyes with localized seeds were saved at 5 years versus 49% with widespread seeds; intravitreal chemotherapy now controls vitreous seeds in most eyes.

Vision depends on where the tumors lie: a tumor at the macula, the center of the retina, can limit sight even after a cure, while peripheral tumors may leave vision largely intact. A child who keeps one healthy eye can develop normally.

Trilateral retinoblastoma

About 5% of children with the heritable form (older studies reported up to 15%) develop a related tumor in the midline of the brain, usually the pineal region, most often between about 20 and 36 months of age. Five-year survival is about 67% when it is found early versus 11% when found late, so some centers perform brain MRI every 6 months for up to 5 years; practice varies.

Second cancers and lifelong follow-up

In high-income countries, where almost every child survives the eye cancer, second cancers and trilateral retinoblastoma have replaced metastasis as the main causes of death among survivors, mainly in the heritable form. The most common are bone tumors (osteosarcoma), soft-tissue sarcomas, melanoma and brain tumors, sometimes decades later. The American Academy of Ophthalmology cites a risk of about 25% within 50 years for heritable survivors even without radiotherapy, and external beam radiotherapy multiplies it by more than three — so unnecessary radiation is avoided and MRI is used instead of CT.

In the first years after treatment, examinations under anesthesia take place about every 6–8 weeks until around age 3, so that any regrowth or new tumor is treated while small. Survivorship care also includes hearing tests after carboplatin, kidney and nerve checks after chemotherapy, fertility counseling and prompt review of persistent bone pain or a new lump in heritable survivors. Keep a written summary of the diagnosis, genetic result and treatments.

Retinoblastoma treatment in Turkey: what families from abroad can expect

If you are considering retinoblastoma treatment abroad, the first step does not require travel. You can send your child's records for a remote second opinion: RetCam photographs and drawings from examinations under anesthesia, the group of each eye, ultrasound and OCT images, MRI as original DICOM files, a treatment summary (drug names, doses, number of cycles or sessions), genetic reports and any pathology report. The reply explains how the findings look, whether the proposed plan seems appropriate and whether an examination in Antalya is advisable; it cannot replace an examination under anesthesia when decisions depend on the exact appearance of the tumors.

The first visit. Most young children need an examination under anesthesia soon after arrival. Your child will need to stop eating and drinking for some hours beforehand, following the anesthesia team's instructions. A parent can usually stay with the child until the anesthetic begins and rejoin them in the recovery area; the anesthesia team explains the arrangements. Afterward, Prof. Türkoğlu discusses the findings and options with you in plain language.

Several visits. Retinoblastoma is rarely treated in a single trip: intra-arterial chemotherapy is given in sessions a few weeks apart, intravitreal injections are repeated at roughly weekly intervals, systemic chemotherapy runs over months, and EUAs are needed every few weeks at first. Expect a longer stay or several visits; some care, such as blood tests during chemotherapy, may be shared with your doctors at home when that is safe.

Travel. Antalya Airport is about 20–30 minutes by car from the practice. See our pages for international patients and eye cancer treatment costs in Turkey.