Orbital metastasis in children linked to poor prognosis

The authors of this study retrospectively reviewed the medical records of all pediatric patients diagnosed with neuroblastoma in Olmstead County, Minn., during a 40-year period ending in December 2008. The incidence of neuroblastoma in this population was 11.8 per million patients younger than 15 years. Ophthalmic involvement was observed in six of the 14 children diagnosed with neuroblastoma during the study period (43 percent). Orbital metastasis occurred in all six of these patients (100 percent) and was associated with poor prognosis.

Other ocular manifestations included proptosis and ecchymosis in four patients (67 percent), ptosis in two (33 percent) and strabismus in one (17 percent). Proptosis and ecchymosis were the presenting sign of neuroblastoma in two cases (14 percent). None of the children had Horner syndrome or opsoclonus.

The survival rate for all 14 children was 57 percent at one year and 50 percent at five years, while the survival rate for the six children with orbital metastasis was 17 percent at nine months and zero percent at one year.

Five of the six patients (83 percent) with ocular involvement were diagnosed during the first 12 years of the study, and none presented during the last 20 years. These five cases all presented with advanced-stage disease, which may explain the greater occurrence of ocular metastasis compared with other studies.