Medication
Two types of radiation therapy used in treating retinoblastoma include: Internal radiation (brachytherapy). During internal radiation, the treatment device is temporarily placed in or near the tumor. Internal radiation for retinoblastoma uses a small disk made of radioactive material.
Procedures
This type of chemotherapy can be given as the initial treatment or be used when the tumor does not respond to a different type of treatment. Intravitreal chemotherapy is also used to treat retinoblastoma that has spread within the eye. In this type of chemotherapy, drugs are injected directly into the eye.
Nutrition
The dose of systemic chemotherapy is usually based on the eye that has more cancer. Patients with cavitary retinoblastoma respond well to treatment and have favorable long-term outcomes, similar to patients with noncavitary retinoblastoma. For information about the treatments listed below, see the Treatment Option Overview section.
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Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. The retina is the nerve tissue that lines the inside of the back of the eye. The retina senses light and sends images to the brain by way of the optic nerve.
How is radiation therapy used to treat retinoblastoma?
What are the different types of chemotherapy for retinoblastoma?
What are the treatment options for cavitary retinoblastoma?
What is retinoblastoma?
What treatment is used for retinoblastoma?
The main types of treatment for retinoblastoma are: Surgery (Enucleation) for Retinoblastoma. Radiation Therapy for Retinoblastoma. Laser Therapy (Photocoagulation or Thermotherapy) for Retinoblastoma.
Which radiation is used in eye treatment?
Radiation therapy uses high-energy x-rays to kill cancer cells. It is a common treatment for eye melanoma.
What chemotherapy is used for retinoblastoma?
The drugs used most often for retinoblastoma are vincristine (Oncovin, Vincasar PFS), carboplatin (Paraplatin), and etoposide (Toposar, VePesid). Depending on the extent of the tumor, a combination of 2 or more drugs may be recommended. All chemotherapy has side effects that occur during treatment.
What is focal therapy for retinoblastoma?
In the management of intraocular retinoblastoma, the term “focal therapy” refers to focal modalities such as laser treatment, cryotherapy, and brachytherapy. They can be used as primary treatment for small tumors or in conjunction with intravenous chemotherapy for larger tumors (i.e., chemoreduction).
Is beta radiation used to treat?
In ophthalmology, beta radiation has been used widely for the treatment of pterygium and is under evaluation for treatment of age-related macular degeneration and for controlling wound healing after glaucoma drainage surgery.
How is beta radiation used in radiotherapy?
Most RIT uses beta decay. In beta decay, a neutron breaks down, changing to a proton and emitting a high-energy electron (beta particle) and raising the atomic number by one without changing the mass number. Given the length of their path, beta emissions are appropriate for treating tumors larger than 0.5 cm.
What cyclophosphamide is used for?
Cyclophosphamide is used to treat cancer of the ovaries, breast, blood and lymph system, and nerves (mainly in children). Cyclophosphamide is also used for retinoblastoma (a type of eye cancer mainly in children), multiple myeloma (cancer in the bone marrow), and mycosis fungoides (tumors on the skin).
Who makes vincristine?
The shortage is related in part to manufacturing delays by Pfizer, which currently is the only maker of vincristine. Teva Pharmaceuticals, which also manufactured vincristine, made a “business decision” to stop producing the drug in July.
What is intravitreal chemotherapy?
(IN-truh-VIH-tree-ul KEE-moh-THAYR-uh-pee) Treatment in which anticancer drugs are injected directly into the vitreous humor (gel-like fluid inside the eye). Intravitreal chemotherapy is used to treat retinoblastoma that has spread to the vitreous humor and has come back or has not gotten better after other treatment.
What is brachytherapy in surgery?
Brachytherapy (plaque radiotherapy) The use of brachytherapy, also known as internal radiation therapy or episcleral plaque radiotherapy, is limited to small tumors. During brachytherapy, a small amount of radioactive material is placed on the outside of the part of the eyeball where the tumor is, and it stays there for several days.
How does IMRT work?
IMRT lets doctors shape the radiation beams and aim them at the tumor from several angles, as well as adjust the intensity (strength) of the beams to limit the dose reaching the nearby normal tissues. This may let the doctor deliver a higher dose to the tumor, while reducing side effects.
What is EBRT radiation?
External beam radiation therapy (EBRT) focuses radiation beams from a source outside the body on the cancer. This was once a common treatment for retinoblastoma. But because of the side effects it can cause, it is now most often used only for cancers that are not well-controlled with other treatments.
How is radiation focused on the eyeball?
The radiation travels a very short distance, so most of it will be focused only on the tumor. The plaque is sewn in place on the eyeball with tiny stitches during a short operation. It is then removed during a second operation several days later.
What is the best treatment for retinoblastoma?
Radiation Therapy for Retinoblastoma. This treatment uses high energy x-rays or particles to kill cancer cells. Radiation therapy is an effective treatment for some children with retinoblastoma. Compared with surgery, it has the advantage of possibly saving vision in the eye. But radiation therapy also has some disadvantages (see Possible side ...
What is protons beam therapy?
Proton beam therapy. Protons are positive parts of atoms. Unlike the x-rays used in standard radiation, which release energy both before and after they hit their target, protons cause little damage to tissues they pass through and then release their energy after traveling a certain distance.
Is retinoblastoma a hereditary cancer?
This is especially important in children with the hereditary form of retinoblastoma, who are already at increased risk for developing other types of cancer. Newer forms of radiation therapy, such as IMRT and proton beam therapy, target the tumor more precisely and spare more normal tissue. This may make some of these side effects less likely ...
What is the treatment for extraocular retinoblastoma?
Treatment options for extraocular retinoblastoma (CNS disease) include the following: Systemic chemotherapy and CNS-directed therapy with radiation therapy. Systemic chemotherapy followed by myeloablative chemotherapy and stem cell rescue with or without radiation therapy.
What is PDQ cancer?
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of retinoblastoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
What is the cell of origin of retinoblastoma?
Maturing cone precursor cells appear to be the cell of origin in human retinoblastoma. [ 1, 2] Microscopically, the appearance of retinoblastoma depends on the degree of differentiation. Undifferentiated retinoblastoma is composed of small, round, densely packed cells with hypochromatic nuclei and scant cytoplasm. Several degrees of photoreceptor differentiation have been described and are characterized by distinctive arrangements of tumor cells, as follows:
What is genetic counseling?
Genetic counseling is an integral part of the management of patients with retinoblastoma and their families, regardless of clinical presentation. Counseling includes a discussion of the main forms of retinoblastoma, which assists parents in understanding the genetic consequences of each form of retinoblastoma and in estimating the risk of disease in family members. [ 19] Counseling also includes guidance towards appropriate screening for both patients and their families, especially if the risk of developing a second primary malignancy is increased.
Where is intraocular retinoblastoma located?
Intraocular retinoblastoma is localized to the eye; it may be confined to the retina or may extend to involve other structures such as the choroid, ciliary body, anterior chamber, and optic nerve head. Intraocular retinoblastoma, however, does not extend beyond the eye into the tissues around the eye or to other parts of the body.
What is the Reese Ellsworth classification system?
Reese and Ellsworth developed a classification system for intraocular retinoblastoma that has been shown to have prognostic significance for maintenance of sight and control of local disease at a time when surgery and external-beam radiation therapy were the primary treatment options. However, developments in the conservative management of intraocular retinoblastoma have made the Reese-Ellsworth grouping system less predictive for eye salvage and less helpful in guiding treatment. [ 7] This grouping system is seldom used and serves largely as a historical reference.
What is the late effect of retinoblastoma?
Treatment of retinoblastoma aims to save the patient's life and uses an individualized, risk-adapted approach to minimize systemic exposure to drugs, optimize ocular drug delivery, and preserve useful vision.
What is the heritable form of retinoblastoma?
A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception.
What is retinoblastoma eye exam?
Key Points. Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma. Retinoblastoma occurs in heritable and nonheritable forms.
How does enucleation work?
Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye.
Why is retinoblastoma not a routine screening?
CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years.
How old is a child diagnosed with retinoblastoma?
The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease.
How often do you check for retinoblastoma?
After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months.
What is the name of the disease where the brain receives light?
Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain.
How does IAC work?
IAC delivers potent chemotherapy directly into the eye during an EUA, via the ophthalmic artery. It is reached by a catheter inserted into the femoral artery. IAC is used to treat medium to large sized intra-ocular retinoblastoma, and should only be used when there is no risk that tumour can spread outside the eye.
What is the best treatment for retinoblastoma?
Surgical removal of the eye is the best treatment to protect the child when retinoblastoma affects only one eye. Surgery is also needed if treatments fail to kill bilateral tumours, or when there is a high risk that the cancer will spread outside the eye.
Is radiotherapy a last resort?
Radiotherapy used to be a standard retinoblastoma treatment, but it has significant long term side effects. In developed countries, it is now primarily a last resort treatment to save a single remaining eye.
Can cryotherapy be given during anesthesia?
Laser, cryotherapy, periocular and intravitreal chemotherapy injections are given during an Exam Under Anaesthesia (EUA). They may be used separately or in combination with other therapies. As primary treatment, they are effective only for small tumours that do not threaten vision.
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