Chemoreduction for Group E Retinoblastoma: Comparison of Chemoreduction Alone Versus Chemoreduction Plus Low-Dose External Radiotherapy in 76 Eyes

• Published in: Ophthalmology (Rochester, Minn.) Vol. 116; no. 3; pp. 544 - 551.e1
• Main Authors: Shields, Carol L., MD, Ramasubramanian, Aparna, MD, Thangappan, Archana, BS, Hartzell, Kimberly, MD, Leahey, Ann, MD, Meadows, Anna T., MD, Shields, Jerry A., MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.03.2009; Elsevier
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Carboplatin - therapeutic use; Child; Child, Preschool; Combined Modality Therapy; Etoposide - therapeutic use; Eye Enucleation; Female; Humans; Infant; Male; Medical sciences; Miscellaneous; Neoplasm Recurrence, Local; Ophthalmology; Radiotherapy, High-Energy; Retinal Neoplasms - classification; Retinal Neoplasms - drug therapy; Retinal Neoplasms - radiotherapy; Retinal Neoplasms - therapy; Retinoblastoma - classification; Retinoblastoma - drug therapy; Retinoblastoma - radiotherapy; Retinoblastoma - therapy; Retinopathies; Retrospective Studies; Treatment Outcome; Tumors and pseudotumors of the eye, orbit, eyelid, lacrimal apparatus; Vincristine - therapeutic use; Visual Acuity; Human; Nervous system diseases; Retinopathy; Low dose; Malignant tumor; Radiotherapy; Eye; Eye disease; Treatment; External; Ophthalmology; Retinoblastoma; Child; Comparative study; Cancer
• ISSN: 0161-6420; 1549-4713
• DOI: 10.1016/j.ophtha.2008.10.014

Purpose To evaluate chemoreduction (CRD) for group E retinoblastoma. Design Retrospective, comparative case series. Participants Seventy-six eyes of 56 patients with group E retinoblastoma were treated with CRD alone or CRD plus low-dose prophylactic external beam radiotherapy (CRD+P-EBR). The CRD included vincristine, etoposide, and carboplatin (6 cycles). The P-EBR was given routinely 2 months after completion of CRD at a suggested dose of 2600 cGy. Therapeutic EBR (T-EBR) was only given at the time of extensive tumor recurrence at a suggested dose of 3800 cGy. Methods Retrospective chart review. Main Outcome Measures Globe salvage. Results Of the 76 eyes, 64 received CRD alone and 12 received CRD+P-EBR. At the 2-year follow-up, globe salvage was achieved in 29 (53%) of 55 eyes in the CRD group and in 10 (91%) of 11 eyes in the CRD+P-EBR group. At 5 years, globe salvage was achieved in 20 (48%) of 42 eyes in the CRD group and in 4 (80%) of 5 eyes in the CRD+P-EBR group ( P = 0.347). Of the 64 eyes in the CRD group, 16 (25%) were salvaged with CRD alone and 13 (20%) with CRD+T-EBR, whereas 22 (34%) were enucleated after CRD alone and 13 (20%) were enucleated after CRD+T-EBR. Of the 12 eyes in the CRD+P-EBR group, 10 (83%) were salvaged with CRD+P-EBR, whereas 2 (17%) were enucleated and none required T-EBR. The median dose for T-EBR was 3800 cGy, and that for P-EBR was 2600 cGy. Eyes treated with CRD+P-EBR showed significantly less recurrence, leading to less chance of enucleation or therapeutic radiotherapy than that for CRD alone ( P <0.001). Visual acuity was 20/100 or better or fix and follow in 9 (32%) of 28 salvaged eyes in the CRD group and in 4 (40%) of 10 in the CRD+P-EBR group. At 5 years, there were no patients in either group with metastasis of pinealoblastoma or who had died. In one patient in the CRD group, a second cancer developed. Conclusions Group E retinoblastoma managed with CRD+P-EBR showed significantly less need for enucleation or therapeutic radiotherapy than eyes treated with CRD alone. These findings merit further study and consideration. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.