An unusual presentation of primitive embryonal tumour of the meninges in a paediatric patient and review of literature

Abstract

Primitive embryonal tumours of the meninges are an exceedingly rare entity and has been predominantly reported in the paediatric population. It poses a unique challenge in establishing the diagnosis as it closely resembles meningioma. This makes the incorporation of sophisticated techniques such as immunohistochemistry, molecular studies, and genetic analysis mandatory to arrive at a confirmative diagnosis. We hereby report a case of a 13-year boy who was diagnosed with primitive embryonal tumour that was initially sought to be a meningioma. Alongside, we have also included a review of literature on this entity focussing on paediatric population, where only a handful of case reports have been published till date. Although these tumours are biologically aggressive, but they tend to have good response rate with treatment. Due to rarity of this entity, consensus on the management guidelines is lacking. However, an acquaintance to this entity is important amongst neuro-oncologists to decrease the possibility of misdiagnosis and improve the cure rates and survival.