Blindness in children with neuroblastoma

Belgaumi, Asim; Kauffman, William M.; Jenkins, Jesse J.; Córdoba, José Carlos; Bowman, Laura C.; Santana, Víctor M.; Furman, Wayne L.

doi:10.1002/(sici)1097-0142(19971115)80:10<1997::aid-cncr18>3.0.co;2-t

Cited by 46 publications

(38 citation statements)

References 25 publications

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“…1 The incidence of orbital metastasis at 12.5% is also in the published range of between 10 and 20%. [2][3][4] Although the male incidence is reported to be slightly higher, 1 the male to female ratio of 2 : 1 in this small study is likely to be due to chance.…”

Section: Discussionmentioning

confidence: 49%

“…[2][3][4] Neuroblastoma originates in the adrenal medulla or the paraspinal sites where sympathetic nervous system tissue is present. Symptoms are due to a tumour mass or bone pain from metastases.…”

Section: Discussionmentioning

confidence: 99%

“…5 Other manifestations include Horner's syndrome, aniscoria, and strabismus. 2 Ophthalmic involvement by definition is Stage 4 disease according to the International Neuroblastoma Staging System. Orbital involvement falls in the high-risk category and therefore aggressive multiagent chemotherapy consisting of very high doses is employed.…”

Section: Discussionmentioning

confidence: 99%

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Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists

Ahmed

Goel

Khandwala

et al. 2005

Eye

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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.

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Section: Discussionmentioning

confidence: 49%

Section: Discussionmentioning

confidence: 99%

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Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists

Ahmed

Goel

Khandwala

et al. 2005

Eye

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“…10 The prominence of metastatic spread of NB to cranial sites has intrigued investigators for 100 years, 11 “raccoon eyes” from ecchymotic proptosis are an ominous hallmark of NB, and large cranial metastases can cause blindness. 12 …”

Section: Introductionmentioning

confidence: 99%

Hyperfractionated Low-Dose (21 Gy) Radiotherapy for Cranial Skeletal Metastases in Patients With High-Risk Neuroblastoma

Kushner

Cheung

Barker

et al. 2009

International Journal of Radiation Oncology*Biology*Physics

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Purpose We present a large experience (73 patients) using a standard radiotherapy (RT) protocol to prevent relapse in cranial sites where measurable metastatic neuroblastoma (NB), which is an adverse prognostic marker, is common. Methods and Materials High-risk NB patients with measurable cranial disease at diagnosis or residual cranial disease post-induction had those sites irradiated with hyperfractionated 21 Gy; a brain-sparing technique was used for an extensive field. Patients were grouped based on response to systemic therapy. Thus, when irradiated, Group 1 patients were in complete remission and Group 2 patients had primary refractory disease. Follow-up was from the start of cranial RT. Results At three years, the 39 Group 1 patients had a progression-free survival (PFS) rate of 51%; control of cranial disease was 79%. Two relapses involved irradiated cranial sites. Two other patients relapsed in irradiated cranial sites 6 and 12 months after a systemic relapse. At three years, the 34 Group 2 patients had a PFS rate of 33%; control of cranial disease was 52%. Group 2 included 19 patients who had residual cranial (+ extracranial) disease. Cranial sites showed major (n=13), minor (n=2), or no response (n=4) to RT; five patients progressed in the cranial RT field at 10-to-27 months. Group 2 also included 15 patients who had persistent NB in extracranial but not cranial sites: two relapsed in irradiated cranial sites and elsewhere at 8 and 14 months. Cranial RT was well tolerated, with no grade ≥2 toxicities. Conclusions Hyperfractionated 21 Gy cranial RT may help control NB and is feasible without significant toxicity in children.

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“…However, cases of widespread metastasis, at times signaled by proptosis, ecchymosis, and other signs of orbital involvement, continue to have a poor prognosis. 5 Ophthalmic manifestations are well documented and include proptosis, 1,6,7 periorbital ecchymosis, 1,6,7 Horner syndrome, 8-11 opsoclonus/myoclonus, 12 ocular motility defects, 1,13 ptosis, 13 and blindness. 6,14 …”

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confidence: 99%

Incidence, Ocular Manifestations, and Survival in Children with Neuroblastoma: A Population-Based Study

Smith

Diehl

Smith

et al. 2010

American Journal of Ophthalmology

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Purpose To determine the incidence, ophthalmic manifestations, and survival among children with neuroblastoma in a defined population. Design Population-based retrospective cohort. Methods The medical records of all pediatric (< 19 years) residents of Olmsted County, Minnesota diagnosed with neuroblastoma from January 1, 1969, through December 31, 2008, were retrospectively reviewed. Results Fourteen children were diagnosed with neuroblastoma as residents of Olmstead County, Minnesota, during the 40-year period, yielding an age- and gender-adjusted incidence of 11.8 (95% confidence interval [CI]: 5.6 -18.0) per million patients < 15 years of age. The calculated incidence for patients presenting before the age of 5 in this cohort was 1 in 5970 children (95% CI: 3920 – 12580 children). The mean age at diagnosis for the 14 study patients was 22.5 months (range, .4 - 42.6 months). Six (43%) of the 14 (95% CI: 18% -71%) had ocular manifestations, including orbital metastasis in 6 (100%), proptosis and ecchymosis in 4 (67%), ptosis in 2 (33%), and strabismus in 1 (17%). The Kaplan-Meier rate of survival for all 14 children was 57% at 1 year (95% CI: 36% – 90%) and 50% at 5 years (95% CI: 30% - 84%), while the 6 with eye findings had a survival rate of 17% at 9 months (95% CI: 3-100%). Conclusions The incidence of neuroblastoma in this population was 11.8 per million patients < 15 years, with ophthalmic involvement observed in 6 (43%) of the 14 study patients. Orbital metastasis in the 6 children in this cohort was associated with poor prognosis.

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Blindness in children with neuroblastoma

Abstract: among pediatric patients, and orbital metastatic disease is not uncommon in these children. Physical signs as a consequence of orbital metastases, such as proptosis

Cited by 46 publications

References 25 publications

Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists

Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists

Hyperfractionated Low-Dose (21 Gy) Radiotherapy for Cranial Skeletal Metastases in Patients With High-Risk Neuroblastoma

Incidence, Ocular Manifestations, and Survival in Children with Neuroblastoma: A Population-Based Study

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