Primary MPNST in Childhood- A Rare Case Report

Malignant peripheral nerve sheath tumour usually occurs between 20-50 years of age, comprising about 5-10% of soft tissue sarcomas. Only 1.7% of them have been reported to occur in children < 5 months of age according to the literature. Here, we are describing 18 mnth old male child presented with a swelling in the lower back. MRI showed a sacrcoccygeal swelling extending to and communicating with CSF at lower sacral level. Birth history of the child was normal with normal apgar score. The histological diagnosis was malignant peripheral nerve sheath tumour. IHC showed focal positivity of GFAP and S100. Primary spinal MPNST in children are rarer. A careful neurological examination is warranted in children. Early diagnosis and referral to multidisciplinary team are important in ensuring the best diagnosis and optimal therapy in this young age.