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Ocular involvement in neuroblastoma: prognostic implications.

M A Musarella, H S Chan, G DeBoer

    Ophthalmology
    |August 1, 1984
    PubMed
    Summary
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    Ophthalmological signs like proptosis, Horner's syndrome, and opsoclonus are key indicators of neuroblastoma in children. Early detection through these eye signs significantly improves survival rates for this childhood cancer.

    Area of Science:

    • Pediatric Oncology
    • Ophthalmology
    • Medical Diagnostics

    Background:

    • Neuroblastoma is a common childhood cancer, with age at diagnosis being the primary prognostic factor.
    • Early detection and surgical resectability are crucial for favorable outcomes in neuroblastoma.
    • Ophthalmological manifestations occur in approximately 20% of neuroblastoma cases.

    Purpose of the Study:

    • To analyze the significance of ophthalmic signs in the diagnosis and prognosis of neuroblastoma.
    • To correlate specific eye signs with tumor location, stage, and patient survival.
    • To emphasize the importance of investigating neuroblastoma in children presenting with ocular symptoms.

    Main Methods:

    • Retrospective review of 405 children diagnosed with neuroblastoma at the Hospital for Sick Children.

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  • Analysis of ophthalmic findings, including proptosis, Horner's syndrome, and opsoclonus.
  • Correlation of ophthalmic signs with tumor characteristics and survival data.

    • Main Results:

    • Ophthalmic involvement was observed in 80 of 405 children (20%).
    • Proptosis/periorbital ecchymosis (orbital metastases) was associated with a 3-year survival rate of 11.2%, often originating from abdominal neuroblastoma.
    • Unilateral Horner's syndrome indicated localized disease with a 78.6% 3-year survival rate.
    • Opsoclonus-myoclonus presented as a sign of occult, localized neuroblastoma with a 100% 3-year survival rate.
    • Girls had a significantly better survival rate (48.7%) than boys (22.4%).

    Conclusions:

    • Ophthalmological signs are critical indicators for neuroblastoma diagnosis and staging.
    • Specific eye signs correlate with tumor location and prognosis, with Horner's syndrome and opsoclonus indicating better outcomes.
    • Children exhibiting these ophthalmic signs warrant thorough and repeated neuroblastoma investigations.