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Clinicopathological Features and Outcome

Adriana Cuenca, MD; Flor Giron, MD; Daisy Castro, MD; Adriana Fandiño, MD; Myriam Guitter, MD; María T. G. de Dávila, MD, PhD; Guillermo Chantada, MD

Arch Ophthalmol. 2009;127(8):1006-1010.

Objective  To describe the clinical and pathological features of patients with retinoblastoma and microscopic scleral invasion.

Methods  We reviewed all pathology slides of patients with microscopic scleral invasion who were included in 3 prospective treatment protocols (1988-2007). All patients received adjuvant chemotherapy (moderately intensive chemotherapy in the first 2 protocols or a more intensive combination in the third one). Only patients with cut-end invasion received orbital radiotherapy.

Results  Thirty-two of 386 patients had enucleated eyes with intrascleral (21 cases) and transscleral (11 cases) invasion. Of these cases, 20 had tumor invading the optic nerve beyond the lamina cribrosa, with 6 of these having tumor at the surgical margin. Sixteen were treated with moderately intensive chemotherapy and 16 received a higher-intensity regimen. Five-year overall survival was 0.77. Seven patients had an extraocular relapse (central nervous system metastasis, n = 4; systemic metastasis, n = 2; and involving the orbit, n = 3, isolated in 1 and combined with central nervous system disease in 2). All patients who had a relapse died. Patients receiving the intensive regimen had a significantly better outcome (P = .007).

Conclusions  Microscopic scleral invasion might be a risk factor for extraocular relapse, and more intensive chemotherapy results in improved survival for these patients.

Author Affiliations: Department of Pediatrics, Universidad Nacional de Colombia, Bogota (Dr Cuenca); Hospital Escuela de Honduras, Universidad Nacional Autonoma de Honduras, Tegucigalpa (Drs Giron and Castro); and Departments of Ophthalmology (Dr Fandiño), Hemato-Oncology (Drs Guitter and Chantada), and Pathology (Dr de Dávila), Hospital JP Garrahan, Buenos Aires, Argentina.

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