Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists

Abstract

Introduction Neuroblastoma is predominantly a tumour of early childhood, which metastasises to the orbits. In such cases, ophthalmologists are involved in the multidisciplinary management. This unique series from a tertiary referral centre is used to elaborate the ophthalmic associations and the ophthalmologist's role in this rare condition. Methods A review of case notes was performed on six patients who presented to the paediatric ophthalmology -oncology liaison service at the Leeds teaching hospitals between 1998 and 2003. The ophthalmic outcome and role of the ophthalmologist were assessed. Results Average age of presentation was 29.8 months (range 15-69 months). Average duration of follow-up was 19.5 months (range 2-58 months). One child died during treatment. Two have completed treatment and are under follow-up. Presenting features of the six children were proptosis in four, periorbital ecchymosis in two, ocular motility restriction in two, and subconjunctival haemorrhage in one. Only one case developed blindness. Conclusions The role of the ophthalmologist in patients with metastatic orbital neuroblastoma can vary from a supportive role to one of active intervention and management of ophthalmic complications. The ophthalmologist is involved in diagnosis and staging as well as monitoring response to treatment of both the primary disease and secondary ophthalmic complications.

Metastatic tumors of the orbit and ocular adnexa

“…Systemic chemotherapy may be helpful in the treatment of orbital and ocular adnexal metastases, especially from chemosensitive tumors, such as small cell lung cancer and neuroblastoma [22 ].…”

A Rare Case of Congenital Orbital Cystic Neuroblastoma in an Infant

“…The age at presentation is usually 15 months or later. Congenital neuroblastoma is rare, and fewer than 100 cases have been reported in the literature [2, 5, 6]. …”

Incidence of pediatric Horner syndrome and the risk of neuroblastoma: A population-based study

“…3,4,12,14,17 With an incidence of approximately 1 in 7000 children younger than 5 years, [18][19][20][21] neuroblastoma is the most common extracranial solid tumor among children younger than 5 years, and rarely appears in children older than 10 years. 21 The absence of neuroblastoma underlying Horner syndrome in this study prompted a review of the Olmsted County medical records of the 21 pediatric patients with diagnoses of neuroblastoma during the same 40-year period, and none were found to have a concurrent Horner syndrome.…”

“…However, other studies have reported minimal correlation between neuroblastoma and pediatric Horner syndrome. 4,5,18,[22][23][24] The relatively high number of occult malignancies among patients with Horner syndrome in some studies can be explained, at least in part, by referral bias. 3,4,[11][12][13][14] Another potential weakness of this study is that some patients may have sought care outside Olmsted County, thereby underestimating the incidence of Horner syndrome.…”