Practical approach to management of retinoblastoma.
OBJECTIVE
To present a simplified approach to management of retinoblastoma using basic clinical features.


METHODS
In a prospective, nonrandomized, single-center clinical trial, 158 eyes of 103 patients with retinoblastoma were managed with 6 cycles of chemoreduction (vincristine sulfate, etoposide, and carboplatin). The eyes were classified according to the Reese-Ellsworth classification and were also grouped on the basis of clinical features as follows: group 1, tumor only; group 2, tumor plus subretinal fluid; group 3, tumor plus focal seeds (3a, focal subretinal seeds; 3b, focal vitreous seeds); group 4, tumor plus diffuse seeds (4a, diffuse subretinal seeds; 4b, diffuse vitreous seeds); and group 5, neovascular glaucoma or invasive retinoblastoma.


METHODS
Treatment success (avoidance of enucleation and external beam radiotherapy).


RESULTS
According to the Reese-Ellsworth classification, chemoreduction was successful in 100% of group Ia, 100% of group Ib, 86% of group IIa, 100% of group IIb, 91% of group IIIa, 100% of group IIIb, 50% of group IVa, 77% of group IVb, 50% of group Va, and 27% of group Vb. There was erratic correlation of the Reese-Ellsworth classification with treatment success. In contrast, the simplified grouping system displayed a smooth, nonerratic correlation for treatment success, with 100% success for group 1, 91% for group 2, 59% for group 3, and 12% for group 4 (group 5 always managed by primary enucleation). When all 6 subcategory groups were analyzed, there was consistent correlation for treatment success of 100% for group 1, 91% for group 2, 68% for group 3a, 54% for group 3b, 17% for group 4a, and 11% for group 4b.


CONCLUSIONS
This practical approach to retinoblastoma using basic clinical features is predictive of treatment success for eyes in which modern conservative therapy for retinoblastoma is used.
Shields
Carol L
CL
Ocular Oncology Service, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107, USA. carol.shields@shieldsoncology.com
Mashayekhi
Arman
A
Demirci
Hakan
H
Meadows
Anna T
AT
Shields
Jerry A
JA
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage / Q000627:therapeutic use*; D002675:Child, Preschool; D005047:Etoposide / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D011446:Prospective Studies; D019572:Retinal Neoplasms / Q000145:classification / Q000188:drug therapy* / Q000473:pathology; D012175:Retinoblastoma / Q000145:classification / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage / Q000627:therapeutic use*
D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin