Primary diffuse leptomeningeal primitive neuroectodermal tumor presenting as chronic meningitis.

Area of Science:

• Neuro-oncology
• Neuropathology
• Pediatric Neurology

Background:

• Primary diffuse leptomeningeal primitive neuroectodermal tumor (pDLNET) is a rare neoplasm.
• pDLNET can present clinically and radiologically mimicking chronic meningitis or neuroinfection.
• Prompt diagnosis is often challenging due to its rarity and overlapping features with common conditions.

Observation:

• This report details a case of a nine-year-old boy initially treated for chronic meningitis and hydrocephalus with antituberculous therapy.
• The patient's clinical course and initial investigations suggested a persistent meningitis.
• Despite empirical treatment, the condition did not resolve, prompting further investigation.

Findings:

• Meningeal biopsy was crucial for definitive diagnosis.
• The biopsy revealed the presence of a primary diffuse leptomeningeal primitive neuroectodermal tumor.
• This confirmed that the patient's symptoms were due to pDLNET, not a typical neuroinfection.

Implications:

• The case underscores the importance of considering rare neoplasms in the differential diagnosis of refractory neuroinfections.
• A high index of suspicion and timely meningeal biopsy are critical for diagnosing pDLNET.
• This highlights the need to re-evaluate treatment strategies for suspected neuroinfections that fail to respond to standard therapies.