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The management of bifocal intracranial germinoma in children
R Al-Mahfoudh1, R Zakaria, E Irvine
1Department of Neurosurgery, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK, rafid@doctors.net.uk.
Insights
Bifocal intracranial germinoma (BFG) in children can be effectively treated with craniospinal radiotherapy, achieving complete cure. Limited radiation with chemotherapy may suffice for non-disseminated cases, potentially avoiding biopsy.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Radiation Oncology
Background:
- Bifocal intracranial germinoma (BFG) affects the pineal and suprasellar regions.
- BFG is highly radiosensitive, but optimal treatment and outcomes in children are not well-defined.
Purpose of the Study:
- To review a single institution's pediatric BFG cases.
- To combine this series with literature data to evaluate management strategies and outcomes.
Main Methods:
- Retrospective review of 4 pediatric BFG cases treated at one institution.
- Literature search for an additional 38 pediatric BFG cases.
- Analysis of treatment modalities including radiotherapy, chemotherapy, and biopsy.
Main Results:
- All patients treated with craniospinal radiotherapy achieved complete cure with no progression at a mean 3-year follow-up.
- Limited radiation (whole ventricle or focal) plus chemotherapy showed comparable outcomes to craniospinal irradiation in non-disseminated cases.
- Biopsy did not alter outcomes in cases with negative tumor markers and characteristic imaging.
Conclusions:
- Biopsy may be unnecessary for pediatric BFG with classic imaging, negative tumor markers, and diabetes insipidus.
- Craniospinal irradiation may be avoidable in cases without spinal dissemination.
- Further long-term data are needed to confirm the efficacy of focal radiotherapy and chemotherapy regimens.
Objectives:
Bifocal intracranial germinoma (BFG) is a tumour of the pineal and suprasellar regions, which is known to be highly radiosensitive. The definitive treatment and outcomes are not well defined, particularly in the paediatric population. We review our series of purely paediatric cases from a single institution and combine them with the limited reports in the literature to determine the results of different management strategies.
Methods:
Four patients were treated at our institution with a median age of 15.3 years. A literature search identified a further 38 paediatric cases with a median age of 12.9 years.
Results:
All four patients had normal serum and CSF tumour markers. One patient had a diagnosis made based on imaging findings of bifocal pineal and suprasellar lesions presenting with diabetes insipidus. Three others underwent biopsy. All had craniospinal radiotherapy, which has led to complete cure with no cases of progression at a mean follow-up of 3 years. The most common treatment modality in published cases is craniospinal irradiation. In the cases reviewed, limited radiation treatments (whole ventricle or focal) combined with chemotherapy regimens yield comparable outcomes where there is no spinal dissemination. Outcomes do not appear to be altered by biopsy in cases with negative tumour markers and characteristic imaging appearances.
Conclusion:
Patients who present with a classic appearance of germinoma, negative tumour markers and diabetes insipidus probably do not require a biopsy to confirm the diagnosis. No evidence of dissemination may obviate the need for craniospinal irradiation, but good quality long-term follow-up data are required to demonstrate the benefits of combined focal radiotherapy and chemotherapy regimes.
