An unusual trigeminal neurinoma

Area of Science:

• Pediatric neurosurgery
• Neurosurgical oncology
• Cranial nerve tumors

Background:

• Trigeminal neurinomas are rare benign tumors originating from the Schwann cells of the trigeminal nerve.
• Pediatric trigeminal neurinomas are exceptionally uncommon, posing unique diagnostic and surgical challenges.
• A family history of neurofibromatosis (NF) increases the risk of developing various nerve sheath tumors, including neurinomas.

Observation:

• An 18-month-old child presented with proptosis, noted since 4 months of age.
• A large trigeminal neurinoma involving the Gasserian ganglion and all three trigeminal nerve divisions was identified.
• The tumor exhibited a plexiform growth pattern, with extension into the ophthalmic division and along the supraorbital nerve.

Findings:

• The case highlights a massive trigeminal neurinoma in a very young child.
• The tumor's extensive involvement of the Gasserian ganglion and its divisions, including the ophthalmic nerve, is a significant finding.
• The plexiform growth pattern suggests a potentially infiltrative nature, despite the benign histology typical of neurinomas.

Implications:

• This case underscores the importance of early recognition and comprehensive evaluation of pediatric cranial nerve tumors.
• Surgical management requires meticulous planning due to the tumor's size, location, and potential involvement of critical structures.
• The presence of a family history of neurofibromatosis warrants genetic counseling and consideration for hereditary tumor syndromes.