Fatal outcome of intracranial embryonal tumor with multiple rosettes: Case report and review of the literature

Abstract

There are three known histological variant within the family of embryonal tumor with multiple rosettes. This family included embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). Here we report a case and performed a comprehensive overview in terms of clinical, pathological, molecular and management outcomes of this rare entity of paediatric brain tumor. Clinically these variants found to have similar characteristics like age (<4 year) associated with highly aggressive nature with reported survival period of 2-3 years. In immunohistochemistry (IHC), most commonly applied markers were synaptophysin, neurofilament protein, Neu-N and glial fibrillary acidic protein (GFAP). Recent data on molecular subgroups of PNETs have led to new insights on diagnosis and treatment of these tumors. Subsequently, LIN28A immunoexpression was identified as a highly specific marker for ETMR. As these tumor having poor prognosis because of aggressiveness in nature, treated as high risk brain tumors. Here we want to report a highly aggressiveness nature of disease, a 6 year old child presented with fever, headache and vomiting. Radiological diagnosis suggestive of left parieto-occipital lesion in brain underwent two time surgery and IHC suggestive of embryonal tumors with multilayered rosettes -WHO grade-IV. He had not responded to treatment and died with overall survival of 2 months.