| Inclusion Criteria: | 1) Only patients who are between and including the ages of 0 to 6 years at study entry are eligible.
2) All diagnosed children with Retinoblastoma with unilateral disease who undergo enucleation as initial therapy. Patients with or without high-risk histopathologic features will be eligible. High-risk features are defined as: a) Posterior uveal invasion (includes choroidal invasion) b) Any degree of concomitant choroid and optic nerve involvement. c) Tumor involving the optic nerve posterior to the lamina cribrosa as an independent finding. d) Scleral invasion e) Anterior chamber seeding. f) Ciliary body infiltration g) Iris infiltration
3) Evidence of choroidal and/or optic nerve invasion past the lamina cribosa ARE ALLOWED. Since pathology may not be available at study entry on an enucleated eye, patients may be entered on study pending final pathology report. (If histology meets exclusion criteria, then patient will be off study.)
4) The individual institution must submit slides for central review as per guidelines and within 21 calendar days after surgery. (If the slides are not shipped within 21 calendar days of surgery, the patient will be ineligible for the study.)
5) Lansky score greater than or equal to 50
6) No prior therapy other than enucleation
7) CT/MRI of the brain: Baseline CT/MRI of the brain to be performed either prior to enucleation or within 5 weeks of enucleation. Patients who will receive chemotherapy should have a baseline CT/MRI performed prior to initiation of chemotherapy.
8) CBC is required prior to enrollment in all patients.
9) Patients with High-Risk features only: Hematologic function defined as: Hemoglobin gm/dl > 8; and ANC >/= 1000/µL; and platelet count >/= 100,000/µL.
10) Patients with High-Risk features only: 1) Creatinine clearance or radioisotope GFR >/= 70 ml/min/1.73 m^2; OR, 2) maximum serum creatinine (md/dL) based on age/gender as follows: 1 month to < 6 months: 0.4 md/dL; 6 months to < 1 yr: 0.5 md/dL; 1 to < 2 yrs: 0.6 md/dL; 2 to < 6 yrs: 0.8 md/dL; 6 to < 10 yrs: 1 md/dL; 10 to < 13 yrs: 1.2 md/dL; 13 to < 16 yrs: Male: 1.5 md/dL; Female: 1.4 md/dL; >/= 16 yrs: Male: 1.7 md/dL; Female: 1.4 mg/dL.
11) Patients with High-Risk features only: Adequate liver function defined as:Total bilirubin </= 1.5 x upper limit of normal (ULN) for age, and SGOT (AST) or SGPT (ALT) < 2.5 x upper limit of normal (ULN) for age.
12) Study enrollment AND pathology slide submission MUST take place within 21 calendar days of enucleation. Patients must be enrolled BEFORE adjuvant chemotherapy begins. The date adjuvant chemotherapy is to start, if chemotherapy is indicated as per protocol, must be no later than 35 calendar days after the date of enucleation. All patients must sign the initial consent form after enucleation, but prior to study enrollment and prior to submission of slides for review.
13) All patients and/or their parents or legal guardians must sign a written informed consent.
14) Subjects, for whom adjuvant chemotherapy is recommended, must also sign the second consent form (chemotherapy arm) prior to initiation of chemotherapy. (Treatment must start within 35 days of enucleation.) |