Abstract
Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies, which affects children and adolescents with only 10 cases has been reported over 33 years old. They have an aggressive behavior and a high tendency for local recurrence and distant metastasis. Here we present a case of supratentorial PNET which occurred in left frontoparietal lobe of a 36 years old female patient with the chief complaint of aphasia for 2 month duration. Aphasia is being reported for the first time as a consequence of a PNET brain tumor. T1-weighted MRI showed a large mass with mixed isointense to hypointense signals. The tumor was completely removed. Histopathologic examination was indicative of a small round cell tumor. Immunohistochemical analysis showed positivity for CD99. Presence of EWSR1 gene rearrangement confirmed the diagnosis. The patient’s aphasia gradually resolved post operation. Six months follow-up showed no evidence of local recurrence or metastasis.