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Retinoblastoma is a tumor of the eye that typically occurs in children less than 6 years old.? The tumor starts in the retina, which is the back of the eye, behind the pupil. In some children both eyes are affected. Retinoblastoma can run in families, and so it is important that parents, siblings, and offspring of a patient with retinoblastoma be evaluated by an ophthalmologist. Some patients with retinoblastoma involving both eyes also have a tumor in their pineal gland deep within their brain. Patients who survive bilateral retinoblastoma as a child are at risk for additional cancers, including osteosarcoma, as they get older.
Signs or symptoms concerning for Retinoblastoma include:
Retinoblastoma Diagnostic Testing and Surgery
After a careful history and physical examination, we typically obtain:?
Based on the results of opthalmologist examination, a clinical group will be assigned to each eye:
Group A:tumor 3 mm or smaller and confined to the retina but not near the optic disc or fovea (the central area of the retina)
Group B: all other tumors still confined to the retina
Group C: tumor starts in the retina but has spread locally to produce fluid under the retina or small tumor seeds in the fluid of the eyeball?
Group D: tumor has spread throughout the retina and eyeball, but not to the lens
Group E: tumor reaches the lens or pupil muscles, may cause glaucoma, may infiltrate the wall of the eyeball
If there is spread outside the eye, then a Stage is assigned:
Therapy is based on whether the tumor involves one or both eyes and on the Stage. Patients are given the opportunity to enroll on Children's Oncology Group therapy protocols or may choose to be treated off protocol with standard of care therapy.
Unilateral, one-sided tumors that have not spread outside the eye:
Bilateral tumors that have not spread outside the eye:
For any eye for which useful vision cannot be salvaged then enucleation will be needed chemotherapy and local control measures described above are offered if these therapies are not successful, then radiation therapy is offered to try to save vision.
Spread outside the eye:
Chemotherapy will be offered, potentially combined with an autologous stem cell transplant to allow use of higher doses
Useful vision is obtained using local control or chemotherapy without radiation in approximately 91% of Group A, 84% of Group B, 60% of Group C, and 30% of Group D eyes. Addition of radiation has the potential to save a subset of Group E eyes.
About 90% of Stage 2 and 40% of Stage 3 patients are cured with chemotherapy. 40% of Stage 4 patients have achieved prolong remission in initial studies using stem cell transplant.
Therapy for Relapse
Patients are followed for recurrence by the opthalmologist with frequent examinations under anesthesia and by history and physical examinations.? MRI of the eyes and brain will be done periodically. Hearing tests, blood counts, and liver and kidney function will be followed if chemotherapy was given.