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Asynchronous bilateral retinoblastoma: the St. Jude Children's Research Hospital experience
J Fontanesi1, C Pratt, D Meyer
1Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Insights
Children diagnosed with unilateral retinoblastoma (RB) require close follow-up, as some develop a second tumor in the other eye. Early detection and treatment of this companion eye RB are crucial for preserving vision and patient survival.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Genetics
Background:
- Retinoblastoma (RB) is the most common primary intraocular malignancy in children.
- While bilateral RB is common, a subset of unilateral RB cases can develop a secondary tumor in the contralateral eye.
Purpose of the Study:
- To analyze the incidence, characteristics, and outcomes of secondary retinoblastoma development in the companion eye of patients initially diagnosed with unilateral RB.
- To evaluate the effectiveness of various treatment modalities for companion eye RB and assess long-term prognosis.
Main Methods:
- Retrospective review of 172 children with retinoblastoma treated between 1962 and 1993.
- Detailed analysis of nine patients who developed retinoblastoma in the previously unaffected eye after initial unilateral diagnosis.
Main Results:
- Nine out of 107 patients with unilateral RB developed companion eye RB, with diagnosis at 3-24 months.
- Risk factors included family history of RB and unilateral multiple tumors, suggesting germinal mutations.
- Treatments for companion eye RB included irradiation, cryotherapy, and chemotherapy, with 14/18 eyes remaining intact and 8/9 patients alive at follow-up.
Conclusions:
- Close surveillance is essential for unilateral RB patients, particularly those with risk factors.
- Prompt treatment of companion eye RB can lead to favorable outcomes, preserving ocular integrity and patient survival.
- Understanding germinal mutations aids in identifying high-risk individuals for RB development.
Abstract:
Between May 1962 and July 1993, 172 children presented at the St. Jude Children's Research Hospital for evaluation and/or treatment of retinoblastoma (RB). Of these, 65 presented with bilateral disease, while 107 had unilateral tumors. Of these 107 patients, nine subsequently developed RB in the unaffected eye. Initial age at diagnosis of these nine patients ranged from 3 weeks to 24 months (median = 2 months); five of the nine had a family history of RB at the time of initial diagnosis and one patient, without a family history of RB, presented with unilateral multiple tumors indicating inheritance of a germinal mutation and increased risk of RB development in the companion eye. Time to development of companion eye RB was 1-61 months postinitial diagnosis. Treatment of the initial eye included enucleation (n = 4), chemotherapy (n = 3), irradiation (n = 7), or a combination of these three modalities. Reese-Ellsworth grouping of the companion eye disease included I A (n = 7), III A (n = 1), and IV A (n = 1). Treatment of the second affected eye included irradiation in seven patients, cryotherapy in four, and chemotherapy in three. No companion eye has required enucleation to date. At last followup, 14/18 eyes remain intact. There have been no cases of metastatic dissemination; however, one patient has developed a second malignant neoplasm outside the field of irradiation. Eight of the nine patients remain alive. This experience reinforces the need for close follow-up of patients diagnosed with unilateral RB, especially those with a family history of RB and those with unilateral multiple tumors.