Primary optic nerve sheath meningioma in children

Area of Science:

• Neuro-oncology
• Ophthalmology
• Pediatric Oncology

Background:

• Primary optic nerve sheath meningioma (ONSM) originates from arachnoid villi cells within the optic nerve sheath.
• Pediatric cases constitute less than 5% of all ONSM, often presenting aggressively.
• Common histopathological subtypes in children include transitional and meningotheliomatous meningiomas.

Purpose of the Study:

• To review the characteristics, treatment, and outcomes of pediatric primary optic nerve sheath meningioma.
• To highlight the aggressive nature and potential for spread of this tumor in young patients.
• To discuss the role of surgical resection and emerging radiation techniques.

Main Methods:

• Review of existing literature on pediatric optic nerve sheath meningioma.
• Analysis of histopathological subtypes and their prevalence in children.
• Examination of treatment modalities including surgery and radiation therapy.

Main Results:

• Transitional (54%) and meningotheliomatous (38%) subtypes are most common in children.
• Surgical excision is the primary treatment, but subtotal resection can lead to tumor spread.
• Neurofibromatosis type 2 is associated in 28% of pediatric cases.
• While aggressive, no deaths are primarily attributed to this tumor.

Conclusions:

• Pediatric ONSM requires aggressive management due to its potential for spread.
• Fractionated, stereotactic radiation is being explored, but long-term follow-up data is limited.
• Multidisciplinary approaches are crucial for managing this rare pediatric tumor.