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Ocular involvement in neuroblastoma: prognostic implications
Insights
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.
- 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.
Abstract:
Neuroblastoma is one of the commonest childhood malignancies. The most important prognostic factor is age at diagnosis; early diagnosis, when the tumor is still localized and surgically resectable, is second in importance. On retrospective review of children seen at the Hospital for Sick Children, ophthalmic involvement was seen in 80 of 405 (20%). The three major eye signs of neuroblastoma, proptosis, Horner's syndrome and opsoclonus, are closely related to the site, stage of tumor, and outcome of the patient. Proptosis or periorbital ecchymosis due to orbital metastases was present in 60 of 80 children (bilaterally in 33). The 3-year survival rate was 11.2%. In 53 of 60 cases with orbital metastases the neuroblastoma originated in the abdomen. Unilateral Horner's syndrome occurred in 14 children, as the presenting sign in 9, related to localized disease in 11 and in a favorable location (cervical or thoracic neuroblastoma) in 8. The 3-year survival rate was 78.6%. Opsoclonus-myoclonus was the presenting sign of occult, localized neuroblastoma in all 9 children in whom it occurred. The 3-year survival rate was 100%. For all presentations, girls had a significantly better survival rate than boys (48.7% vs. 22.4%). Children presenting with any of these ophthalmological signs should undergo thorough and repeated investigations searching for neuroblastoma.