Some clinical findings at presentation can predict high risk pathology features in unilateral retinoblastoma

GUILLERMO L CHANTADA MD(1), ANDREA GONZALEZ MD(1), ADRIANA FANDINO MD(2), MARIA TG DE DAVILA MD PHD(3), GRACIELA DEMIRDJIAN MD(4), MARCELO SCOPINARO MD(1) AND DAVID ABRAMSON MD(5)

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Año: 2009

1077-4114

Aims: To identify clinical features at presentation in order to help in detecting patients with retinoblastoma and pathology risk factors (PRF) preoperatively and therefore selecting a high risk population that could benefit from preoperative treatment. Methods: Retrospective analysis of a prospectively filled form of 182 consecutive patients with unilateral retinoblastoma treated with initial enucleation form 1988 to 2006. . Univariate and multivariate analyses were done. Major choroidal invasion and postlaminar optic nerve and scleral extension were considered PRF. Within this subgroup, a higher risk cohort (microscopical residual disease caused by invasion transscleral or invasion to the resection margin of the optic nerve) was analyzed separately Results: 164 patients had completely resected and 18 microscopical residual disease 73 had at least 1 PRF (massive invasion to the choroid in 25, to the postlaminar optic nerve in 41, intra-scleral in 10, to the resection margin of the optic nerve in 12 and trans-scleral in 6). 71 patients had glaucoma and 19 buphthalmia. Intraocular pressure, glaucoma and buphthalmia correlated significantly with the occurrence of both PRF and microscopical residual disease in multivariate analysis. Buphthalmia was the most specific factor but the sensitivity was lower. Glaucoma and buphthalmia had a high negative predictive value. Conclusions: Patients presenting with glaucoma and-or buphthalmia have a significantly higher risk for the occurrence of PRF including those resulting in microscopically residual disease.