What is the treatment?

Treatment of Retinoblastoma Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Children with retinoblastoma should have their treatment planned by a team of doctors with expertise in treating cancer in children. Late effects of cancer treatment may include physical problems; changes in mood, feelings, thinking, learning or memory; and second cancers (new types of cancer.) Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments.

Six types of standard treatment Enucleation – surgery to remove the eye and part of the optic nerve. This is done if the tumor is large and there is little or no chance that vision can be saved. The patient will be fitted for an artificial eye after this surgery. Radiation therapy – Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, plaques, or catheters that are placed directly into or near the cancer. – Intensity-modulated radiation therapy (IMRT): A type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of radiation therapy causes less damage to healthy tissue near the tumor.

– Stereotactic radiation therapy: Radiation therapy that uses a rigid head frame attached to the skull to aim high-dose radiation beams directly at the tumors, causing less damage to nearby healthy tissue. It is also called stereotactic external-beam radiation, stereotactic radiosurgery, and stereotaxic radiosurgery. This procedure does not involve surgery. – Proton beam radiation therapy: Radiation therapy that uses protons made by a special machine. A proton is a type of high- energy radiation that is different from an x-ray. – Plaque radiotherapy: Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation.

Cryotherapy Cryotherapy is a treatment that uses an instrument to freeze and destroy abnormal tissue, such as carcinoma in situ. This type of treatment is also called cryosurgery. Photocoagulation Photocoagulation is a procedure that uses laser light to destroy blood vessels to the tumor, causing the tumor cells to die. Photocoagulation may be used to treat small tumors. This is also called light coagulation. Thermotherapy Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball, or using ultrasound, microwaves, or infrared radiation (light that cannot be seen but can be felt as heat). Chemotherapy

For the Patient Chemotherapy- initial Enucleation- if unsuccessful

Medical Chemotherapy – Preservation of normal vision – Primary neoadjuvant chemotherapy or chemoreduction has been the most significant recent advance in the treatment of retinoblastoma. – Prophylactic chemotherapy is recommended if a tumor is in the optic nerve past the lamina cribrosa because these cases have a poor survival prognosis. – Use of neoadjuvant chemotherapy has the advantage of limiting the necessity for EBRT and reducing the possibility of EBRT-related complications.

Surgical Enucleation – Enucleation is performed when there is no chance of preserving useful vision in an eye. Patients generally requiring enucleation are those who present with total retinal detachments and/or the posterior segment is full of the tumor, in which case it is clear the patient cannot retain any form of useful vision.