Retinoblastoma

Retinoblastoma (RB) is the most common intraocular cancer in children, with 400–500 cases diagnosed in the U.S. each year. It is extremely rare in adults.

There are two types of retinoblastoma:

Hereditary: Typically affects both eyes (10% of cases).

Non-Hereditary: Usually affects only one eye.

Although RB is genetic, only 6% of newly diagnosed patients have a family history. Genetic counseling is recommended for children with germ-line mutations to guide future care.

RB is graded A through E, with A being the least advanced:

Group A: Can often be treated with laser therapy or cryotherapy.

Groups B–D: Traditionally treated with systemic chemotherapy, which may have side effects.

Group D: Historically had a ~50% failure rate with systemic chemotherapy.

Group E: Eye removal was often necessary.

Comprehensive Care

Bascom Palmer Eye Institute works closely with Sylvester Comprehensive Cancer Center, providing advanced care for retinoblastoma. Sylvester is the only center in the Southeastern U.S. and one of few in the nation highly skilled in intra-arterial chemotherapy, which delivers chemotherapy directly to the eye, reducing systemic side effects and improving eye preservation rates.

Diagnostic Tests

Early, accurate diagnosis is critical. Our specialists may use:

Retinal Examination

Retinal Examination Ophthalmoscope is used to examine the retina while pupils are dilated.

Optical Coherence Tomography (OCT)

Provides 3-D imaging of retinal structures.

Ultrasound

High-frequency sound waves produce detailed images of the eye and socket.

Fluorescein Angiography

Dye is injected into a vein to visualize retinal blood vessels.

Electroretinogram (ERG)

Measures electrical responses of retinal cells.

Bone Scan

Detects if cancer has spread to bones or the skull.

umbar Puncture (Spinal Tap)

Checks cerebrospinal fluid for cancer cells if the optic nerve is involved.

Bone Marrow Aspiration/Biopsy

Performed if cancer is suspected to have spread into the blood or marrow.

Treatment Options

Intra-Arterial Chemotherapy

A thin catheter delivers chemotherapy directly to the ophthalmic artery.
Young patients with C–E graded tumors benefit from reduced systemic side effects.
Typically requires 2–3 treatments spaced three weeks apart.
Only ~5% fail to respond; additional local injections can target remaining cancer cells in the eye.

Cryotherapy

Other Options

A thin catheter delivers chemotherapy directly to the ophthalmic artery.
Young patients with C–E graded tumors benefit from reduced systemic side effects.
Typically requires 2–3 treatments spaced three weeks apart.
Only ~5% fail to respond; additional local injections can target remaining cancer cells in the eye.

Why Choose Bascom Palmer Eye Institute?

National-Leading Partnership

Multidisciplinary Care Teams

Advanced Eye-Sparing Treatments

Top-Ranked Prosthetic Options

Research & Innovation

With early diagnosis and specialized care, children with retinoblastoma have a high chance of eye preservation and cure.

FAQ

What is retinoblastoma, and how does it present in young children?

This rare eye cancer arises in retinal cells, often genetic (RB1 mutation), striking under-5s with a white pupil glow (leukocoria) in photos or strabismus. Bilateral in hereditary (40%), it grows fast but 99% curable early—think rogue fireworks in the retina, dimming young lights if unchecked.

How is retinoblastoma screened and confirmed?

Red reflex tests at checkups flag asymmetry; ultrasound/MRI sizes tumors, genetics confirm heritable. Staging groups A-E guides globe salvage—biopsy rare, as enucleation pathology suffices.

What multi-modal treatments cure retinoblastoma?

Chemo shrinks intra-arterial for precision, laser/cryo ablates smalls, plaque radiation spares tissue. Advanced: enucleation with chemo. 95% survival, vision varies—trials test immunotherapy.

What genetic counseling follows retinoblastoma diagnosis?

RB1 testing siblings/parents; carriers get lifelong MRI surveillance. Fertility preservation pre-chemo. Empowers families, turning fear to proactive plans.

What long-term follow-up do survivors need?

Annual exams track second cancers (osteosarcoma risk), cardiac from anthracyclines. Psychosocial support blooms resilience; most thrive, eyes or orbits adapted beautifully.