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Volume 116, Issue 3, Pages 544-551.e1 (March 2009)


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

Presented as a poster at: American Academy of Ophthalmology Annual Meeting, November 2008, Atlanta, Georgia; and will be presented as paper at: International Society of Ocular Oncology Meeting, September 2009, Cambridge, England.

Carol L. Shields, MD1Corresponding Author Informationemail address, Aparna Ramasubramanian, MD1, Archana Thangappan, BS1, Kimberly Hartzell, MD1, Ann Leahey, MD2, Anna T. Meadows, MD2, Jerry A. Shields, MD1

Received 5 August 2008; received in revised form 9 October 2008; accepted 10 October 2008. published online 20 January 2009.

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.

Available online: January 20, 2009.

1 Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania

2 Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Corresponding Author InformationCorrespondence: Carol L. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107

 Manuscript no. 2008-938.

 Statistical analysis provided by Rishita Nutheti, Hyderabad, India. Carol L. Shields, MD, has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

 Support provided by the Retina Research Foundation (Charles L. Schepens Lecture) of the Retina Society in Cape Town, South Africa (CLS); the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (JAS); a donation from Michael, Bruce, and Ellen Ratner, New York, New York (JAS, CLS); and the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (CLS, JAS). The funders had no role in the design and conduct of the study; in the collection, analysis, and interpretation of the data; or in the preparation, review, or approval of the manuscript.

 Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

PII: S0161-6420(08)01071-3

doi:10.1016/j.ophtha.2008.10.014


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