Patients should be monitored for follow-up of the primary tumor. The majority of recurrences appears within 3 years of diagnosis and recurrences are extremely rare after 5 years of age. The follow-up schedule is largely dependent on the therapy used and the extent of original disease. For example, children who are treated with neoadjuvant chemotherapy and local ophthalmic therapies, who retain their eye and are
Table 23-5. Potential Preparative Chemotherapy Regimens for Stem Cell Transplant for Metastatic Retinoblastoma"
Carboplatin-etoposide-cyclophosphamide Carboplatin 250-350 mg/m2/day on days 1-5 Etoposide 350 mg/m2 on days 1-5 Cyclophosphamide 1.6 g/m2/day on days 2-5
Carboplatin 500 mg/m2/day or dosed to attain an area under the curve of 7 mg/min/mL using the Calvert formula on days 1-3 Thiotepa 300 mg/m2/day on days 1-3 Etoposide 250 mg/m2 on days 1-3
All are followed by infusion of autologous stem cells.
"Many alternative preparative regimens are available and no current data to support any one as a standard of care.
not treated with external beam radiotherapy, require closer follow-up than children who have undergone an enucleation and for whom pathology failed to show any high-risk features.
For patients treated with chemotherapy for intraocular or extraocular disease, an evaluation under anesthesia (EUA) is recommended to be performed every 3-4 weeks until there is no active tumor seen on a minimum of three EUAs, then every 6-8 weeks until age 3 years, then every 4-6 months to age 10 years. Patients should be transitioned to an office exam without anesthesia when old enough to cooperate.
For patients who have undergone an enucleation and/or received external beam radiotherapy, an evaluation should be performed 4-6 weeks post-treatment, then every 2-3 months for the first year post-therapy, every 3-4 months the following year, every 6 months until age 5 years, and then annually. For patients with extraoc-ular disease, assessment for recurrent metastatic disease should also be performed, which should include CT or MRI of the brain and orbits, CSF evaluation, bone marrow evaluation, and bone scan.
To screen for trilateral retinoblastoma, patients diagnosed with bilateral retinoblas-toma, especially those diagnosed at less than 1 year of age, or those with positive family history are recommended to have a head CT or MRI every 6 months from the end of therapy, continuing until 5 years of age.
Was this article helpful?