Primitive neuroectodermal tumor involving the frontal skull base in an infant

Area of Science:

• Pediatric neurosurgery
• Pediatric oncology
• Neuro-oncology

Background:

• Primitive neuroectodermal tumors (PNETs) are rare, aggressive central nervous system neoplasms.
• Frontal skull base PNETs in infants are exceptionally uncommon.
• Early diagnosis and surgical intervention are crucial for management.

Observation:

• A 5-month-old male infant presented with rapidly progressing left-sided exophthalmos.
• Magnetic resonance imaging revealed a 6 cm intradural, extra-axial frontal mass.
• The mass was well-demarcated and enhanced after contrast administration.

Findings:

• Surgical resection achieved complete tumor removal.
• Pathological examination confirmed the diagnosis of primitive neuroectodermal tumor (PNET).
• Intradural extra-axial PNET in this location and age group is exceedingly rare.

Implications:

• This case highlights the importance of considering rare diagnoses in pediatric neuro-oncology.
• Complete surgical excision is the primary treatment modality.
• The prognosis for intradural extra-axial PNETs remains guarded due to their rarity and aggressive nature.