Please also see the following pages for more detailed information.
Adjuvant
A therapy used in addition to, or accompanying another treatment.
Autologous Stem Cell / Bone Marrow Transplant
A procedure used to “rescue” a patient’s bone marrow system following super high dose chemotherapy. Stem cells or bone marrow are removed from the patient, and returned after high-dose chemotherapy to destroy the cancer. The transplanted cells produce new blood and immune cells to replace the bone marrow destroyed by intensive chemotherapy.
Biopsy
Surgery to remove a sample of tumour for pathology analysis. Due to the risk of spreading tumour outside the eye, biopsy is never done to confirm retinoblastoma. Pathology can be done safely only after surgical removal of the whole eye.
Bilateral Retinoblastoma
Childhood eye cancer in both eyes. The cancer does not spread from one eye to the other – tumours arises from unique retinal cells in each eye.
Brachytherapy (radioactive plaque)
Tiny pellets containing radioactive material are sewn onto the surface of the eye above the tumour, while the child is under general anaesthetic. They are removed during a second surgery after several days.
Broviac
A form of Central Venous Catheter used to deliver chemotherapy and other drugs, and draw blood samples. A thin flexible tube is surgically implanted into a large vein in the chest, and the other end exits the chest, held securely in place under the skin by a special cuff. The Broviac is the same as a Hickman Line, but has a smaller diameter and smaller lumen. The catheter usually has two lumens (tubes) outside the body, but may have three. The catheter requires meticulous daily care to prevent infection.
Central Venous Catheter (CVC) / Central Venous Access Device (CVAD)
A thin flexible tube surgically inserted into a large vein in the chest, for delivery of systemic chemotherapy. The other end of the catheter may either be outside the body (Broviac or Hickman Line) or attached to a small device just under the skin (Port-a-Cath / Mediport).
Cerebrospinal Fluid (CSF)
A clear fluid bathing the brain and spinal cord.
Chemotherapy
Drug treatment of cancer. Several drugs are usually used in combination for retinoblastoma. These may be delivered by infusions into a vein or artery, by injection or taken in the form of tablets. Treatment usually lasts for a number of months, with several days of treatment followed by several weeks of rest before the next cycle.
Child Life
Preparation, education, distraction and psychological supports based on natural child development, designed to help the child cope with serious illness and other traumatic life events, and improve their quality of life.
Constitutional RB1 Pathogenic Variant
A change in DNA sequence of the RB1 gene that may cause retinoblastoma and can be passed to the next generation. This genetic change may have been inherited from a parent or occurred at conception or early in foetal development.
Cryotherapy / Cryosurgery
A procedure done during EUA in which extreme cold is applied to the tumour through a probe placed on the surface of the eye.
Enucleation
Surgical removal of an eye.
EUA - Examination Under Anaesthesia
An eye examination carried out under general anaesthetic to enable thorough observation of the retina in a young child.
Extraocular Retinoblastoma
Retinoblastoma that has spread beyond the eye to the orbit, optic nerve, brain, bone marrow or other parts of the body..
Fluroscopy
An imaging technique used to map the arteries during delivery of intra-arterial chemotherapy. A continuous x-ray beam is passed through the body, and images are transmitted to a screen to assist the surgeon in guiding a catheter through the child’s body from the leg to the eye.
Hickman Line
A form of Central Venous Catheter used to deliver chemotherapy and other drugs, and draw blood samples. A thin flexible tube is surgically implanted into a large vein in the chest, and the other end exits the chest, held securely in place under the skin by a special cuff. The Hickman Line is the same as the Broviac, but has a larger diameter and larger lumen. The catheter usually has two lumens (tubes) outside the body, but may have three. The catheter requires meticulous daily care to prevent infection.
Informed Consent
An individual willingly agrees to participate in a specific activity, or allows his/her child to be involved in that activity, after first being advised of the need, process, benefits and risks.
International Intraocular Retinoblastoma Classification (IIRC)
A retinoblastoma staging system (A-E) that predicts outcome from chemotherapy and focal therapy.
Intra-Arterial Chemotherapy (IAC) / Ophthalmic Artery Chemotherapy (OAC)
A procedure delivering chemotherapy directly into the eye through the ophthalmic artery, via a catheter inserted in the child’s femoral artery (in the leg).
Intrathecal Chemotherapy
Drugs are injected directly into the cerebrospinal fluid, fluid surrounding the brain and spinal cord. This may be done during a lumbar puncture, or via an ommaya reservoir.
Intravenous Chemotherapy
Chemotherapy administered through a vein.
Intravitreal Chemotherapy (IViC)
Adjuvant chemotherapy administered during EUA, injected directly into the vitreous behind the lens, through the sclera. On removing the needle, the injection site is treated with cryotherapy to manage risk of cancer cells escaping the eye.
Laser / Photocoagulation
Focused beams of light are delivered through an ophthalmoscope during an EUA, killing cancer cells by destroying their blood supply.
Leukocoria
A white reflection in the eye. In retinoblastoma, this is caused by the cancer preventing light from reaching the retina, so it is reflected back as a pearly-white glow, like a cat’s eye caught in the headlights of a car.
Monocular
Having only one seeing eye.
Metastatic Retinoblastoma
Cancer that has spread from the eye to the brain, bone marrow or other parts of the body.
Multifocal
More than one tumour in one eye. This strongly indicates the child’s risk for a constitutional RB1 mutation, but differentiating between true multifocal tumour and large seeds can be very difficult.
Neutropaenia
An absolute Neutrophil count (ANC) below 1,000, when a child is at high risk of severe infection.
Ommaya Reservoir
A small port surgically implanted under the skin on the head. A fine tube is threaded from the port into a ventricle (open space) in the brain through which cerebrospinal fluid flows. Chemotherapy can be delivered and CSF samples drawn out via the port.
Ophthalmoscopy
Examination of the inside of the eye, using either a device mounted on the doctor’s head, or a hand-held device to examine the eye.
Pathology
Microscopic examination of surgically removed tissue (such as an eye) to confirm diagnosis, determine extent of the cancer, risk of relapse and need for further treatment.
Periocular Chemotherapy (subtenon / subconjunctival)
Injection of chemotherapy agents into tissues surrounding the eye, done during EUA. The needle does not penetrate the eye itself and chemotherapy is absorbed into the eye through the sclera and cornea. Injections may be subtenon or subconjunctival.
- Subtenon: injection of chemotherapy agents into the space behind the eye.
- Subconjunctival: injection under the mucus membrane that coats the eye and lines the eyelids.
Port-a-Cath
A small device surgically implanted just under the skin, connected to a large vein in the chest via a thin flexible tube. When the port’s septum is accessed using a special needle, chemotherapy and other drugs can be delivered and blood samples taken repeatedly. The port generally offers less discomfort than repeated needle sticks and less risk than an external Hickman Line / Broviac.
Post Laminar Optic Nerve Invasion (PLONI)
Cancer that has spread past the lamina cribrosa, a plate of connective tissue at the point where the optic nerve exits the eye.
Proton Beam Therapy
A form of external beam radiation using protons.
Radiotherapy / External Beam Radiotherapy
Treatment with ionizing radiation. High-energy rays are used to damage cancer cells and stop them from growing and dividing.
Radioactive Plaque (brachytherapy)
Tiny pellets containing radioactive material are sewn onto the surface of the eye above the tumour, while the child is under general anaesthetic. They are removed during a second surgery after several days.
Relapse / Recurrence
Cancer that becomes active again after a period of remission.
Remission
No detectable living cancer.
Reese-Ellsworth Classification (REC)
A retinoblastoma staging system (1a – 5b) that predicts outcome from external beam radiotherapy treatment.
Second Primary Cancer
Cancers other than retinoblastoma that can occur in carriers of a RB1 pathogenic vairant, and children treated with chemotherapy or radiotherapy.
Seeds
Seeds are tiny tumour fragments that brake off the larger mass and float into nearby fluids inside the eye. Seeds can float between the retina and choroid (“subretinal seeds”) or into the gel filling the eye (“vitreous seeds”).
The larger the tumour is, and the faster it grows, the more likely it is to break apart and allow the cancer to spread to other parts of the eye. Retinoblastoma can also escape from the eye and spread to distant sites in the body via uncontrolled seeding into the blood, lymphatic system or cerebro-spinal fluid (fluid bathing the brain).
Potential to save the eye decreases when seeds are present. Any seeds are hard to treat, and focal treatments such as laser and cryotherapy cannot be applied to single, invisible cells, until they grow.
New therapies in clinical study can deliver chemotherapy directly to seeds in the vitreous and under the retina, so doctors hope to have more success in safely saving children’s eyes with seeds that do not threaten life.
Stem Cell Rescue
A procedure used to “rescue” a patient’s bone marrow system following super high dose chemotherapy. Stem cells are removed from the patient’s blood, and returned after high-dose chemotherapy given to destroy the cancer. The transplanted cells produce new blood and immune cells to replace the bone marrow destroyed by the intensive chemotherapy.
Subretinal Seeds
Tiny cancerous fragments that have broken away from a tumour and floated into fluid between the retina and the choroid.
Systemic Chemotherapy
Chemotherapy that travels around the entire body. For retinoblastoma, this is usually administered through a vein, but may be given orally.
TNMH (Tumour, Node, Metastesis, Heritability) classification
A cancer staging system developed, monitored and enforced by the American Joint Commision on Cancer and the Union for International Cancer Control (AJCC/UICC). Many cancers have a unique TNM classification, including retinoblastoma. Retinoblastoma is the first cancer to include a Heritability (H) classification.
Tonometry
The process of measuring pressure within the eye. A machine that measures eye pressure is called a tonometer.
Trilateral Retinoblastoma
A rare form of heritable retinoblastoma that causes a malignant tumour to form in the brain.
Ultrasound
A form of imaging used to examine the eye without the risks of invasive surgery. After administering anaesthetic drops, a small imaging probe coated sterile gel is placed on the surface of the eye. High frequency sound pulses penetrate the eye and bounce off different structures within to produce a pattern of echoes that are received by a computer and translated into a visual image. The B Scan, used in retinoblastoma care, produces cross section images of the eye.
Unifocal
One tumour in the eye
Unilateral Retinoblastoma
Tumour{s) affecting only one eye.
Vitreous Seeds
Tiny fragments that have broken off a tumour and invaded the vitreous humor, the clear, jelly-like substance filling the middle of the eye.
