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Marita S. Uusitalo, MD, PhD; Kurtis R. Van Quill; Ingrid U. Scott, MD, MPH; Katherine K. Matthay, MD; Timothy G. Murray, MD; Joan M. O'Brien, MD

Arch Ophthalmol. 2001;119:41-48.

Objectives  To identify risk factors for metastatic disease on histopathologic specimens of enucleated eyes from patients with unilateral retinoblastoma, and to evaluate the value of chemoprophylaxis in preventing disease dissemination.

Methods  Medical records from patients with unilateral retinoblastoma who underwent primary enucleation were reviewed at the University of California, San Francisco (1977-1998) and Bascom Palmer Eye Institute, University of Miami, Miami, Fla (1991-1998). All routine histopathologic specimens were reexamined. The extent of tumor invasion into the optic nerve or ocular coats and the prescribed chemoprophylactic regimen were recorded.

Results  This retrospective study included 129 patients followed for a median of 54 months. Three patients had tumor invading the sclera. The optic nerve was involved to some extent in 82 patients, 11 of whom had tumor extension beyond the lamina cribrosa. The surgical margin of the optic nerve was involved in an additional 4 patients. The choroid was involved in 43 patients, and was considered massively affected in 12 patients. Anterior segment involvement was observed in 10 patients. Postenucleation chemoprophylaxis was administered to 4 of 4 patients who had tumor cells at the surgical margin of the optic nerve and to 7 of 11 patients with postlaminar disease, all of whom had at least 1 mm of postlaminar tumor extension. External beam radiotherapy was administered to 3/4 and 1/11 of these patients, respectively. Chemoprophylaxis was not administered to patients with choroidal or anterior chamber involvement unless the optic nerve was also involved beyond the lamina cribrosa. One patient with tumor extending to the surgical margin of the optic nerve died of metastatic disease.

Conclusions  Chemoprophylaxis is necessary for patients with tumor extending to the surgical margin of the optic nerve and is likely to be beneficial in preventing metastases in patients with tumor extending beyond the lamina cribrosa. We did not offer chemoprophylaxis to patients with prelaminar optic nerve disease or isolated choroidal involvement, and these patients remained free of disseminated disease.

From the Division of Ocular Oncology, Department of Ophthalmology (Drs Uusitalo and O'Brien and Mr Van Quill) and the Division of Pediatric Clinical Oncology, Department of Pediatrics (Dr Matthay), University of California, San Francisco; the Ophthalmic Pathology Laboratory, Department of Ophthalmology, Helsinki University Central Hospital, Helsinki, Finland (Dr Uusitalo); and the Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Fla (Drs Scott and Murray).

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