This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (12)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Ocular/ Adnexal Tumors  • Drug Therapy  • Drug Therapy, Other  • Alert me on articles by topic  Social Bookmarking   What's this?

Carol L. Shields, MD; Abdallah Shelil, MD; Jacqueline Cater, PhD; Anna T. Meadows, MD; Jerry A. Shields, MD

Arch Ophthalmol. 2003;121:1571-1576.

Objective  To evaluate the occurrence of new retinoblastomas in patients treated with 6 cycles of chemoreduction.

Design  Prospective nonrandomized single-center case series.

Setting  Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, Philadelphia, Pa, in conjunction with the Division of Oncology at The Children's Hospital of Philadelphia.

Participants  A total of 162 eyes of 106 patients with retinoblastoma treated with 6 cycles of chemoreduction between January 1, 1995, and May 31, 2002.

Intervention  All patients received intravenous chemoreduction with vincristine sulfate, etoposide, and carboplatin, combined with focal treatment (cryotherapy or thermotherapy) to each retinal tumor.

Main Outcome Measure  Development of new intraretinal retinoblastoma during or after treatment with chemoreduction. Recurrent subretinal tumor seeds or vitreous seeds were excluded from this analysis, and only primary new intraretinal tumors were included.

Results  Of 28 patients with unilateral retinoblastoma, new intraretinal tumor development was found during or after chemoreduction in 2 (9%) of the 23 patients with sporadic disease and 4 (80%) of the 5 patients with familial disease. The new tumor was located in the macula in none, between the macula and equator in 7 (54%), and between the equator and ora serrata in 6 (46%). Of the 78 patients with bilateral retinoblastoma, new tumor development was found during or after chemoreduction in 11 (19%) of the 57 patients with sporadic disease and 8 (38%) of the 21 patients with familial disease. The new tumor was macula in 2 (4%), between the macula and equator in 30 (55%), and between the equator and ora serrata in 23 (42%). Overall, according to Kaplan-Meier analysis, new tumor development occurred in 23% of patients by 1-year follow-up and 24% by 5-year follow-up. By multivariate analysis, the most important risk factors for the development of new tumors was younger age at presentation (median age, 2 months with new tumor vs 9 months without new tumor) and family history of retinoblastoma (12 [48%] of patients with new tumor vs 14 [17%] without new tumor).

Conclusions  Children with retinoblastoma treated with chemoreduction should be followed for new intraretinal tumor development, as it peaks at a mean interval of 5 months after initiation of chemoreduction and affects 24% of patients by 5 years of follow-up. New tumors are most commonly found in those who develop disease as young infants and those with a family history of retinoblastoma.

From the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa (Drs C. L. Shields, Shelil, Cater, and J. A. Shields), and Division of Oncology, The Children's Hospital of Philadelphia (Dr Meadows). The authors have no relevant financial interest in this article.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Retinoblastoma: Review of Current Management
Chintagumpala et al.
The Oncologist 2007;12:1237-1246.
ABSTRACT | FULL TEXT  

Macular Retinoblastoma Managed With Chemoreduction: Analysis of Tumor Control With or Without Adjuvant Thermotherapy in 68 Tumors
Shields et al.
Arch Ophthalmol 2005;123:765-773.
ABSTRACT | FULL TEXT