| "Do not go where the path may lead, go instead where there is no path - and leave a trail." ~ Ralph Waldo Emerson ~ |
| Reprinted from OPHTHALMOLOGY, Vol. 105, No. 4, April 1998 Published by Lippincott-Raven Publishers Printed in U.S.A. Copyright 0 1998 by the American Academy of Ophthalmology, Inc. Objective. This study aimed to investigate the relationship in bilateral retinoblastoma survivors between the incidence of second tumors and the age when external beam radiation (EBR) was used. Design: A retrospective analysis of patients diagnosed with retinoblastoma was performed by examining records for background information and treatment information as well as reviewing documentation of patients with second monocular tumors. Two telephone interviews were conducted for follow-up as well as inquiries directed to tumor registries and state databases. Participants: The original study included 1729 patients treated in New York and Boston; the current study includes only the 1506 patients treated in New York. Of those, 816 patients were diagnosed with bilateral retinoblastoma, had sufficient treatment data to be useful, and survived at least 1 year from diagnosis. Main Outcome Measures: The subjects were observed for evidence of the development of second monocular tumors. Results: There was a significant decrease in tumor-free survival among patients treated with EBR before the age of 12 months, but no significant difference between the group treated with EBR after the age of 12 months and the group not treated with EBR. For tumors in the field of radiation, patients treated with early EBR showed a significant decrease in tumor-free survival when compared to patients treated with late EBR, with no significant difference between late radiation and no radiation. There were no significant differences between groups for tumors out of the field of radiation. Significant differences attributable to the use of EBR were found only for tumors of the skull and face bones and for tumors of the soft tissue of the head. Conclusions: The long-term effect of radiation treatment on survivors of bilateral retinoblastoma is to increase the incidence and affect the distribution of second tumors. However, no increased risk is observed for tumors out of the field of radiation among patients who underwent radiation, and the risk for tumors in the field of radiation is heavily dependent on the age at which EBR is given and may be acceptably small to the patient after the age of 12 months. It is now generally accepted that survivors of bilateral retinoblastoma and of unilateral retinoblastoma who presumably carry a germline RBI mutation on the basis of a positive family history of retinoblastoma are at high risk of developing a variety of second primary neoplasms.' ' It has further been shown that the external beam radiation therapy often used to treat retinoblastoma, particularly bilateral retinoblastoma, can increase that risk significantly.1-6 As a result, although the use of chemotherapy in intraocular retinoblastoma is not new,9`1 there has been a recent resurgence of interest in various modern chemotherapeutic protocols,12-14 as well as a general attempt to avoid the use of radiation therapy in favor of focal methods. In large populations exposed to low doses of radiation in childhood, such as atomic bomb survivors, Marshall Islanders exposed to radioactive fallout, or residents of the Chernobyl area, an inverse relationship between age at exposure and risk of patients developing thyroid cancer has been well described.1` A similar relationship, indicating that younger patients are more sensitive to the tumorigenic effects of ionizing radiation, also has been found in the development of breast cancer in females exposed to low-dose radiation.21,22 However, we are unaware of any study of retinoblastoma survivors that explicitly takes into account the age of the patient at treatment. The current study attempts to examine this issue and to illuminate the effect of age at radiation treatment on the subsequent risk of second monocular tumors. ___ Ophthalmology 1998,,105.,573-580 Originally received: July 18, 1997. Revision accepted: October 20, 1997. From the Department of Ophthalmology, New York Hospital-Cornell Medical Center, New York, New York. Presented in part at the International Symposium on Ocular Tumors, Jerusalem, Israel, April 6-10, 1997; at the Cornell Ophthalmology Alumni Meeting, New York City, May 2, 1997; and at the Association for Research in Vision and Ophthalmology Meeting, Ft. Lauderdale, Florida, May 11-16, 1997. Reprint requests to David H. Abramson, MD, 70 East 66th Street, New York, NY 10021 Go back to ORbIT MEDICAL ARTICLES page. |
| ORPHANS OF THE CANCER STORM |
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| Second Nonocular Tumors in Survivors of Bilateral Retinoblastoma: A Possible Age Effect on Radiation-related Risk. David H. Abramson, MD, Christopher M. Frank, AB |