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Ectopic intracranial retinoblastoma in childhood
Insights
Ectopic intracranial retinoblastoma developed in 12 children, often spreading within the central nervous system and proving fatal. Early, comprehensive treatment targeting the whole neuraxis is crucial for potential cure.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Neuro-oncology
Background:
- Retinoblastoma is a malignant tumor of the retina in children.
- Ectopic intracranial retinoblastoma is a rare but serious complication.
Purpose of the Study:
- To analyze the incidence, characteristics, and outcomes of ectopic intracranial retinoblastoma.
- To evaluate treatment strategies and survival rates for this condition.
Main Methods:
- Retrospective review of 630 children with retinoblastoma treated over 30 years.
- Analysis of tumor location, patient demographics, treatment modalities, and survival data.
Main Results:
- Twelve children (1.9%) developed ectopic intracranial retinoblastoma, primarily in the pineal (8) or suprasellar (4) regions.
- Median time to intracranial involvement was 34 months; median survival post-diagnosis was 8 months.
- Central nervous system metastasis was the most common cause of death.
Conclusions:
- Ectopic intracranial retinoblastoma, though rare, has a poor prognosis with current treatments.
- Adequate therapy to the entire neuraxis and high-dose radiotherapy to the primary tumor are essential for potential cure.
Abstract:
Twelve out of a series of 630 children with retinoblastoma, treated in the ocular oncology units at St Bartholomew's and Moorfields Eye Hospitals during the past 30 years, have developed ectopic intracranial retinoblastoma. The ectopic tumour occurred in the pineal region in eight children and in the suprasellar region in four. Ten patients had bilateral retinoblastoma, one unilateral disease, and one child presented with an isolated suprasellar tumour but no evidence of retinal disease. The interval from the initial diagnosis of retinoblastoma to the development of ectopic intracranial disease ranged from 4 to 70 months, median 34 months. Methods of treatment for the ectopic tumour varied, but all 12 children died with a median survival of only 8 months following the diagnosis of ectopic retinoblastoma. Subsequent spread of tumour to other sites within the central nervous system proved to be the most frequent cause of death. Ectopic intracranial retinoblastoma is a potentially curable neoplasm, but it requires adequate therapy to the whole neuraxis as well as high dose equivalent radiotherapy to the primary tumour.