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Retinoblastoma with micrometastasis to CSF
M L Gimblett1, P C Wellings, M Lewis
1Department of Ophthalmology, Wellington Hospital, New Zealand.
Pathology
|January 1, 1995
Summary
This case highlights the critical role of cerebrospinal fluid (CSF) cytology from the Sylvian fissure in diagnosing retinoblastoma metastasis. Early detection via this method significantly improved patient prognosis.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Neuro-oncology
Background:
- Retinoblastoma, a pediatric eye cancer, can involve the optic nerve and metastasize.
- Accurate staging is crucial for effective treatment and prognosis.
Observation:
- A 28-month-old male presented with squint and leukocoria, diagnosed with unilateral retinoblastoma with optic nerve involvement.
- Standard metastasis workup, including lumbar puncture cytology, was negative.
- Surgical excision of the optic nerve was performed, with histopathology clear at the resection margin.
Findings:
- Cerebrospinal fluid (CSF) cytology from the Sylvian fissure, obtained during surgery, revealed malignant retinoblastoma cells.
- This finding contrasted with the negative lumbar puncture cytology, indicating its limitations in detecting CSF micrometastasis.
Implications:
- CSF cytology from the Sylvian fissure is vital for detecting retinoblastoma spread, altering management and prognosis.
- A neurosurgical approach allows extensive optic nerve resection and direct CSF sampling.
- Intrathecal chemotherapy, intravenous chemotherapy, and cranial radiotherapy were administered, leading to a favorable outcome.