Embryonal Tumour with Multi-layered Rosettes; a rare paediatric C.N.S tumour. A case report and review of literature.

Abstract

Introduction: Embryonal tumours with multi-layered rosettes (ETMR) are rare small  round blue cell tumour of the central nervous system. They are one of the  most aggressive brain tumours usually encountered in infants and young  children. Classified as WHO grade 4 tumours. Case Summary: A 5 year Old male patient presented with a progressive vision impairment  with reported bumping into edges, global headaches associated with emesis,  a progressive hemiparesis (power of upper limb 3/5 and a lower limb of 2/5)  and a Right Facial nerve palsy. M.R.I findings: A left Parieto-Occipital single large lobulated mass with  perilesional vasogenic oedema. Craniotomy and a Gross Total Resection performed with an improvement in  symptoms. Histopathological findings: WHO grade 4 lesion composed of primitive  undifferentiated neuroepithelial cells (small round blue cells; see fig. 2.0 for  illustration) . In keeping with the Classic Supra-tentorial PNET and molecular  Amplicon at 19q13.42 studies recommended (hence not tested). Conclusion: ETMRs are rare and highly aggressive tumours that pose significant  diagnostic and therapeutic challenges, particularly in young children.  • Their rapid progression and poor prognosis underscore the importance of  early detection, which can be facilitated by recognizing characteristic  imaging features.