Cavitary Changes in Retinoblastoma: Relationship to Chemoresistance
Received 28 May 2004; accepted 10 January 2005. published online 09 May 2005.
Objective
To describe the clinical features and outcome of treatment of retinoblastomas presenting with ophthalmoscopically visible cavities.
Design
Retrospective, noncomparative, interventional case series.
Participants
Sixteen eyes of 15 patients harboring retinoblastomas with ophthalmoscopically visible cavities treated between February 1995 and July 2003.
Methods
Patient demographics, eye and tumor characteristics, and response to treatment were recorded.
Main Outcome Measures
Decrease in tumor size and tumor recurrence.
Results
The mean patient age at diagnosis was 20 months. The mean number of retinoblastomas per eye was 2 (range, 1–6). Associated subretinal fluid was graded as absent in 5 eyes, minimal in 6 eyes, mild in 4 eyes, and severe in 1 eye. None of the tumors had vitreous seeds, and 5 (31%) had subretinal seeds. Ophthalmoscopically visible calcification occupied a mean of only 3% of the tumor (median, 0%; range, 0%–30%). Intratumoral cavities were characteristically visible in the superficial portion of the tumor and had a mean diameter of 2.0 mm (median, 1.5; range, 0.5–5.0). The mean number of cavities per tumor was 2 (median, 1; range, 1–5). Three tumors were treated with enucleation, 1 with observation, and 12 with chemoreduction. Two months after chemoreduction, the mean reduction in tumor base was 19% (median, 16%; range, 0%–42%), and the mean reduction in tumor thickness was 18% (median, 12%; range, 0%–59%). During a mean follow-up of 32 months (median, 23; range, 12–58), none of the cavitary retinoblastomas recurred or gave rise to vitreous or new subretinal seeds.
Conclusion
Retinoblastomas containing ophthalmoscopically visible cavitary spaces do not show a substantial decrease in size after chemotherapy. Cavitary changes in retinoblastoma may be a sign that the tumor is well differentiated and may impart a better prognosis.
Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania
Reprint requests and correspondence to Carol L. Shields, MD, Oncology Service, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107.
Manuscript no. 240414.
Supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania; the Award of Merit in Retina Research, Houston, Texas (JAS); the Rosenthal Award of the Macula Society, Cleveland, Ohio (CLS); and the Macula Foundation, New York, New York (CLS).