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Systemic adjuvant chemotherapy for advanced malignant ocular medulloepithelioma
Ibrahim H N Sheriff1, Esin K Karaa2, Tanzina Chowdhury1,3
1Retinoblastoma Service, Department of Ophthalmology, The Royal London Hospital, London, UK.
Eye (London, England)
|February 2, 2022
Summary
Adjuvant chemotherapy using vincristine, etoposide, and carboplatin appears safe and effective for advanced ocular medulloepithelioma (diktyoma). This treatment may prevent metastatic spread in paediatric patients with malignant features.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Medical Oncology
Background:
- Ocular medulloepithelioma (diktyoma) is a rare, potentially malignant pediatric tumor originating from the non-pigmented ciliary epithelium.
- Adjuvant chemotherapy indications and regimens for advanced cases are not well-defined.
- This study aimed to evaluate the treatment benefit of adjuvant chemotherapy in advanced ocular medulloepithelioma.
Purpose of the Study:
- To determine if adjuvant chemotherapy provides a treatment benefit for advanced ocular medulloepithelioma.
- To assess the safety and efficacy of a specific chemotherapy regimen in preventing metastatic spread.
Main Methods:
- Retrospective case series of pediatric patients with advanced ocular medulloepithelioma treated at a specialist ocular oncology center.
- Patients underwent enucleation and some received adjuvant systemic chemotherapy with vincristine, etoposide, and carboplatin.
- Outcomes analyzed included histopathology, recurrence, metastases, and survival.
Main Results:
- Four male patients (mean age 31 months) were included.
- Three patients (75%) received adjuvant chemotherapy due to malignant histopathological features.
- No recurrence, metastases, or tumor-related death were observed during a mean follow-up of 81.5 months.
Conclusions:
- This is a unique series reporting the management of advanced malignant ocular medulloepithelioma with adjuvant vincristine, etoposide, and carboplatin.
- The regimen appears safe and potentially effective in preventing metastatic spread.
- Further research is warranted to define optimal indications and regimens for adjuvant chemotherapy.
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