Chemoreduction Followed by Local Therapy and Adjuvant Chemotherapy for Advanced Intraocular Retinoblastoma: A Pilot Study in a Single Center

Abstract

Intraocular (IO) retinoblastoma (RB) has traditionally been treated with enucleation (ENU) or external beam radiotherapy (EBRT). Recently, clinical trials are in progress to cure RB without ENU or EBRT in order to salvage the globe and to avoid unacceptable side effects of EBRT. We performed a pilot study to treat patients with advanced Reese-Ellsworth (RE) stage IO RB with initial chemotherapy (CRx) followed by local therapy (LT) and adjuvant CRx. Ten eyes (8 RE group V, 2 RE group IV) from 9 patients were enrolled from March 2001 to November 2001. All tumors responded to CRx. In 5 of 10 eyes, the RB was enough to be treated with LT after chemoreduction. One patient who underwent LT is waiting for ENU due to post-cryotherapy complication. For a median follow-up of 13 months (8-16 mo), 4 eyes that received LT and adjuvant CRx were relapse-free. A patient with bilateral RB who failed to be a candidate for LT was rescued with high-dose CRx and hematopoietic stem cell transplantation. Consequently, by treating patients according to our strategy, we were able to salvage 6 out of 10 eyes without ENU or EBRT. These results suggest that chemoreduction followed by LT and adjuvant CRx might offer the opportunity to salvage the globe and vision even in patients with advanced stage IO RB.

Effect of Intravenous Chemotherapy Regimen on Globe Salvage Success Rates for Retinoblastoma Based on Disease Class—A Meta-Analysis

“…Of these 2710 studies, 93 studies met the initial inclusion criteria, reporting on the treatment of intraocular retinoblastoma in human patients with IV CRD therapy. Of these 93 studies, 27 [ 4 , 5 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 ] included sufficient data on eye classification and outcomes to be included in this meta-analysis ( Figure 1 and Table 1 ). The median number of eyes included in these studies was 43 (range 10–249 eyes).…”

“…The median number of eyes included in these studies was 43 (range 10–249 eyes). Eight studies [ 4 , 31 , 32 , 43 , 44 , 45 , 47 , 48 ] used enhanced CRD (either higher doses, additional drugs, or a greater number of treatment cycles), 12 studies [ 5 , 30 , 33 , 34 , 35 , 36 , 37 , 38 , 40 , 41 , 42 , 49 ] used two-drug regimens, and 12 studies [ 5 , 29 , 32 , 33 , 39 , 44 , 45 , 46 , 50 , 51 , 52 , 53 ] used standard CRD with 4–6 cycles of standard-dose VEC. In total, 23 studies [ 4 , 5 , 29 , 30 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 47 , …”

“…In total, 23 studies [ 4 , 5 , 29 , 30 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 47 , 48 , 49 , 52 , 53 ] reported R-E tumor classifications, while 10 studies [ 31 , 38 , 43 , 46 , 47 , 48 , 49 , 50 , 51 , 52 ] reported ICRB/IIRC tumor classifications, and 6 studies [ 38 , 43 , 47 , 48 , 49 , 52 ] classified eyes according to both the R-E and ICRB/IIRC classification systems. Two studies [ 39 , 43 ] were restricted to only reporting on R-E Group V eyes, while 1 study [ 44 ] was limited to only reporting R-E Group IV or V eyes. Similarly, 2 studies [ 50 , 51 ] were limited to only reporting ICRB Group D eyes.…”

“…Similarly, 2 studies [ 50 , 51 ] were limited to only reporting ICRB Group D eyes. Thus, 5 studies reported on success rates for only more advanced eyes [ 39 , 43 , 44 , 50 , 51 ] ( Table 1 ).…”

“…A second analysis was performed based on individual patient level data. Of all 93 studies that met our inclusion criteria, sufficient individual patient level data were reported in 14 studies [ 4 , 30 , 31 , 32 , 33 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 ]. Of these 14 studies, all used R-E classification except for one [ 43 ], which reported eyes using the ICRB classification system.…”