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A rare presentation of pediatric germinoma mimicking optic pathway glioma: illustrative case
Stenia Accilien1, William S Coggins1, Deanna Sasaki-Adams1
1Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
Insights
This case report highlights a rare suprasellar germinoma that mimicked an optic pathway glioma. Accurate diagnosis is crucial due to differing treatment protocols for these pediatric brain tumors.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Neuroradiology
Background:
- Intracranial germinomas are rare pediatric tumors, typically in the pineal or suprasellar regions.
- Hypothalamic-optic pathway gliomas are common, often pilocytic astrocytomas along the visual pathway.
- Germinomas respond well to radiation and chemotherapy, unlike gliomas which may require observation or targeted therapies.
Purpose of the Study:
- To report a rare case of suprasellar germinoma presenting as an optic pathway glioma.
- To emphasize the importance of considering germinoma in the differential diagnosis of midline suprasellar lesions involving the optic pathway.
- To underscore the necessity of obtaining adequate tissue for definitive diagnosis due to treatment disparities.
Main Methods:
- Case report detailing a patient with a suprasellar mass.
- Initial transsphenoidal biopsy suggested optic pathway glioma.
- Subsequent pterional biopsy confirmed suprasellar germinoma.
Main Results:
- Radiographic and intraoperative findings initially mimicked an optic pathway glioma.
- Histopathological analysis via pterional biopsy definitively diagnosed a suprasellar germinoma.
- This case illustrates a rare presentation of germinoma.
Conclusions:
- Suprasellar germinomas can radiographically mimic optic pathway gliomas.
- Accurate tissue diagnosis is critical for appropriate management of these rare pediatric brain tumors.
- Multidisciplinary consideration is essential for midline suprasellar lesions involving the optic pathway.
Background:
Intracranial germinomas are rare pediatric tumors classically occurring in the pineal region, the infundibular recess, and septal region. Hypothalamic-optic pathway gliomas are typically pilocytic astrocytomas that can occur at any point along the optic pathway or within the hypothalamus. Germinomas are highly radiosensitive and chemotherapy-sensitive with an overall good prognosis after treatment. In contrast, hypothalamic-optic pathway gliomas are typically managed with observation if asymptomatic and treated with chemotherapy or targeted therapies if they are symptomatic or progressive.
Observations:
Suprasellar germinomas are a known alternative location; however, suprasellar germinomas extending along the optic pathway are exceedingly rare. Neurosurgeons, neuroradiologists, and neuro-oncologists should consider germinomas when crafting a differential around a midline suprasellar lesion involving the optic pathway. Given the differences in treatment of a germinoma versus an optic pathway glioma, obtaining sufficient tissue for a diagnosis is paramount in these rare cases with radiographic equipoise.
Lessons:
The authors report a case of a suprasellar germinoma mimicking an optic pathway glioma in its radiographic appearance. The intraoperative appearance of the lesion during the attempted transsphenoidal biopsy appeared consistent with an optic pathway glioma. Subsequent biopsy via a pterional approach confirmed the diagnosis of germinoma. https://thejns.org/doi/10.3171/CASE24804.
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