Abstract
Ewings sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) belongs to the family of malignant small and blue round cell tumors. It usually occurs in children and young adults with 3/4th of the cases arising from bone and 1/4 from soft tissue. Here, we present two cases of intracranial ES/pPNET who presented with mass effect. Management consists of surgical excision followed by adjuvant chemotherapy. Intracranial ES/pPNETs are highly aggressive and rare malignancies, reported to comprise of 0.03% of all intracranial tumors. The most common genetic aberration associated with ES/pPNET is chromosomal translocation t (11,12) (q24;q12). Patients with intracranial ES/pPNETs may present in acute or delayed manner. The presenting symptoms and signs depend on the location of the tumor. Intracranial pPNET although slow growing, they are highly vascular and may present as neurosurgical emergencies due to mass effect. We have presented the acute presentation of this tumor and its management.
Copyright
Association for Helping Neurosurgical Sick People. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-Non Derivative-Non Commercial License, permitting copying and reproduction so long as the original work is given appropriate credit.
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This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License, which permits unrestricted reproduction and distribution, for non-commercial purposes only; and use and reproduction, but not distribution, of adapted material for non-commercial purposes only, provided the original work is properly cited.
