Abstract
BACKGROUND: Intracranial Ewing's sarcoma (ES) is a rare entity with <15 cases reported in the literature. It belongs to a family of round-cell neuroectodermally derived tumors bearing many similarities to peripheral primitive neuroectodermal tumor (pPNET). There is currently no established treatment protocol. Reported cases are treated with either surgery alone or surgery with adjuvant chemotherapy and radiation. CASE DESCRIPTION: We describe a case of intracranial left frontal ES in a 19-year-old patient who presented with change in behavior. Diagnosis was unclear based on radiological findings on MRI and CT alone. MRI brain with contrast demonstrated a large extra-axial ovoid heterogeneously enhancing left frontal convexity mass. The patient underwent gross total resection with adjuvant chemotherapy and radiation. No local or systemic recurrence was found at 12 months postoperatively. CONCLUSION: Intracranial ES/pPNET is rare tumor with nonspecific clinical presentation and radiological findings. They are locally invasive. Surgery with adjuvant chemoradiation is the mainstay treatment. Distinction of pPNET and cPNET is important for therapeutic and prognostic purposes.