Primary MPNST in Childhood- A Rare Case Report

Area of Science:

• Pediatric Oncology
• Neuro-oncology
• Soft Tissue Sarcomas

Background:

• Malignant peripheral nerve sheath tumors (MPNST) are rare soft tissue sarcomas, typically affecting adults aged 20-50.
• Pediatric MPNST, especially primary spinal tumors, are exceptionally uncommon, with <1.7% reported in infants under 5 months.

Observation:

• An 18-month-old male presented with a lower back swelling.
• MRI revealed a sacrococcygeal mass extending into the cerebrospinal fluid (CSF) space at the lower sacral level.
• Histopathological diagnosis confirmed MPNST, with immunohistochemistry showing focal GFAP and S100 positivity.

Findings:

• This case represents a rare instance of primary spinal MPNST in a young child.
• The tumor's extension into the CSF space presents unique diagnostic and therapeutic challenges.

Implications:

• Early and thorough neurological examination is crucial for children presenting with unexplained swellings.
• Prompt diagnosis and multidisciplinary team management are vital for optimizing outcomes in pediatric MPNST.